GlyCosmos Portal

Homo sapiens (human)

Summary

Taxonomy ID: 9606
PubChem Taxonomy: 9606

Genes (1582) Glycans (5831) Glycoproteins (18496) Lectins (298) Pathways (2090)

Displaying entries **1801 - 1825** of **2090** in total

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Pathway Name	Protein Name	UniProt ID	Gene Symbol ▼	GlyTouCan ID
Regulation of Complement cascade	B-lymphocyte antigen CD19 C3a anaphylatoxin chemotactic receptor C4b-binding protein alpha chain C4b-binding protein beta chain C5a anaphylatoxin chemotactic receptor 1 C5a anaphylatoxin chemotactic receptor 2 CD59 glycoprotein CD81 antigen Carboxypeptidase B2 Carboxypeptidase N catalytic chain Carboxypeptidase N subunit 2 Clusterin Complement C1q subcomponent subunit A Complement C1q subcomponent subunit B Complement C1q subcomponent subunit C Complement C1r subcomponent Complement C1s subcomponent Complement C2 Complement C3 Complement C4-A Complement C4-B Complement C5 Complement component C6 Complement component C7 Complement component C8 alpha chain Complement component C8 beta chain Complement component C8 gamma chain Complement component C9 Complement decay-accelerating factor Complement factor B Complement factor H-related protein 1 Complement factor H-related protein 2 Complement factor H-related protein 3 Complement factor H-related protein 4 Complement factor H-related protein 5 Complement factor H Complement factor I Complement receptor type 1 Complement receptor type 2 IGHV7-81 protein Immunoglobulin heavy constant gamma 1 Immunoglobulin heavy constant gamma 2 Immunoglobulin heavy constant gamma 3 Immunoglobulin heavy constant gamma 4 Immunoglobulin heavy variable 1-2 Immunoglobulin heavy variable 1-46 Immunoglobulin heavy variable 1-69 Immunoglobulin heavy variable 2-5 Immunoglobulin heavy variable 2-70 Immunoglobulin heavy variable 3-11 Immunoglobulin heavy variable 3-13 Immunoglobulin heavy variable 3-23 Immunoglobulin heavy variable 3-30 Immunoglobulin heavy variable 3-33 Immunoglobulin heavy variable 3-48 Immunoglobulin heavy variable 3-53 Immunoglobulin heavy variable 3-7 Immunoglobulin heavy variable 3-9 Immunoglobulin heavy variable 4-34 Immunoglobulin heavy variable 4-39 Immunoglobulin heavy variable 4-59 Immunoglobulin kappa constant Immunoglobulin kappa variable 1-12 Immunoglobulin kappa variable 1-16 Immunoglobulin kappa variable 1-17 Immunoglobulin kappa variable 1-33 Immunoglobulin kappa variable 1-39 Immunoglobulin kappa variable 1-5 Immunoglobulin kappa variable 1D-12 Immunoglobulin kappa variable 1D-16 Immunoglobulin kappa variable 1D-33 Immunoglobulin kappa variable 1D-39 Immunoglobulin kappa variable 2-28 Immunoglobulin kappa variable 2-29 Immunoglobulin kappa variable 2-30 Immunoglobulin kappa variable 2D-28 Immunoglobulin kappa variable 2D-30 Immunoglobulin kappa variable 2D-40 Immunoglobulin kappa variable 3-11 Immunoglobulin kappa variable 3-15 Immunoglobulin kappa variable 3-20 Immunoglobulin kappa variable 3D-20 Immunoglobulin kappa variable 4-1 Immunoglobulin kappa variable 5-2 Immunoglobulin lambda constant 1 Immunoglobulin lambda constant 2 Immunoglobulin lambda constant 3 Immunoglobulin lambda constant 6 Immunoglobulin lambda constant 7 Immunoglobulin lambda variable 1-40 Immunoglobulin lambda variable 1-44 Immunoglobulin lambda variable 1-47 Immunoglobulin lambda variable 1-51 Immunoglobulin lambda variable 2-11 Immunoglobulin lambda variable 2-14 Immunoglobulin lambda variable 2-23 Immunoglobulin lambda variable 2-8 Immunoglobulin lambda variable 3-19 Immunoglobulin lambda variable 3-1 Immunoglobulin lambda variable 3-21 Immunoglobulin lambda variable 3-25 Immunoglobulin lambda variable 3-27 Immunoglobulin lambda variable 6-57 Immunoglobulin lambda variable 7-43 Immunolglobulin kappa light chain variable region L22 (Fragment) Membrane cofactor protein Neutrophil elastase Plasma protease C1 inhibitor Prothrombin UGa8H (Fragment) V1-11 protein (Fragment) V1-13 protein (Fragment) V1-16 protein (Fragment) V1-20 protein (Fragment) V1-3 protein (Fragment) V1-5 protein (Fragment) V1-7 protein (Fragment) V1-9 protein (Fragment) V2-11 protein (Fragment) V2-15 protein (Fragment) V2-17 protein (Fragment) V2-19 protein (Fragment) V2-8 protein (Fragment) V3-2 protein (Fragment) V3-3 protein (Fragment) V3-4 protein (Fragment) V4-1 protein (Fragment) V4-2 protein (Fragment) V4-6 protein (Fragment) V5-1 protein (Fragment) V5-4 protein (Fragment) V5-6 protein (Fragment) Vitamin K-dependent protein S Vitronectin	A0A075B6P5 A0A075B6S6 A0A0C4DH25 A0A0C4DH73 A0M8Q6 A2KUC3 A2NJV5 A2NXD2 P00734 P00736 P00751 P01024 P01031 P01593 P01594 P01597 P01599 P01601 P01602 P01611 P01614 P01615 P01619 P01624 P01699 P01700 P01701 P01703 P01704 P01705 P01706 P01709 P01714 P01715 P01717 P01718 P01721 P01742 P01743 P01762 P01763 P01764 P01766 P01767 P01768 P01772 P01780 P01782 P01814 P01817 P01824 P01825 P01834 P01857 P01859 P01860 P01861 P02745 P02746 P02747 P02748 P04003 P04004 P04211 P04430 P04432 P04433 P05155 P05156 P06310 P06312 P06315 P06331 P06681 P07225 P07357 P07358 P07360 P08174 P08246 P08603 P09871 P0C0L4 P0C0L5 P0CF74 P0CG04 P0DOY2 P0DOY3 P10643 P10909 P13671 P13987 P15169 P15391 P15529 P17927 P20023 P20851 P21730 P22792 P23083 P36980 P60033 P80748 Q02985 Q03591 Q16581 Q5NV61 Q5NV62 Q5NV64 Q5NV65 Q5NV66 Q5NV67 Q5NV68 Q5NV69 Q5NV75 Q5NV79 Q5NV80 Q5NV81 Q5NV82 Q5NV83 Q5NV84 Q5NV85 Q5NV86 Q5NV87 Q5NV89 Q5NV90 Q5NV91 Q5NV92 Q6PIL0 Q92496 Q96IY4 Q9BXR6 Q9P296	ACBP APOJ AZ3B BF BFD C1IN C1NH C1QA C1QB C1QC C1QG C1R C1S C2 C3 C3AR1 C3BR C3DR C3R1 C4A C4B C4BP C4BPA C4BPB C4B_2 C5 C5AR C5AR1 C5AR2 C5L2 C5R1 C6 C7 C8A C8B C8G C9 CD19 CD46 CD55 CD59 CD81 CFB CFH CFHL CFHL1 CFHL1P CFHL2 CFHL3 CFHL4 CFHL5 CFHR1 CFHR1P CFHR2 CFHR3 CFHR4 CFHR5 CFI CLI CLU CO4 CPAMD1 CPAMD2 CPAMD3 CPAMD4 CPB2 CPN1 CPN2 CR CR1 CR2 DAF ELA2 ELANE F2 FHR1 FHR2 FHR3 FHR4 FHR5 GPR77 HF HF1 HF2 HFL1 HFL2 HFL3 HNFAG09 IF IGHG1 IGHG2 IGHG3 IGHG4 IGHV IGHV1-2 IGHV1-46 IGHV1-69 IGHV2-5 IGHV2-70 IGHV3-11 IGHV3-13 IGHV3-23 IGHV3-30 IGHV3-33 IGHV3-48 IGHV3-53 IGHV3-7 IGHV3-9 IGHV4-34 IGHV4-39 IGHV4-59 IGHV7-81 IGKC IGKV1-12 IGKV1-16 IGKV1-17 IGKV1-33 IGKV1-39 IGKV1-5 IGKV1D-12 IGKV1D-16 IGKV1D-33 IGKV1D-39 IGKV2-28 IGKV2-29 IGKV2-30 IGKV2D-28 IGKV2D-30 IGKV2D-40 IGKV3-11 IGKV3-15 IGKV3-20 IGKV3D-20 IGKV4-1 IGKV5-2 IGLC1 IGLC2 IGLC3 IGLC6 IGLC7 IGLV IGLV1-40 IGLV1-44 IGLV1-47 IGLV1-51 IGLV2-11 IGLV2-14 IGLV2-23 IGLV2-8 IGLV3-1 IGLV3-19 IGLV3-21 IGLV3-25 IGLV3-27 IGLV6-57 IGLV7-43 KUB1 MCP MIC10 MIC11 MIN1 MIN2 MIN3 MSK21 PROS PROS1 SERPING1 TAPA1 TSPAN28 V1-11 V1-13 V1-16 V1-20 V1-3 V1-5 V1-7 V1-9 V2-11 V2-15 V2-17 V2-19 V2-8 V3-2 V3-3 V3-4 V4-1 V4-2 V4-6 V5-1 V5-4 V5-6 VTN
Peroxisomal lipid metabolism	2-Hydroxyacid oxidase 2 Acyl-CoA-binding domain-containing protein 4 Acyl-CoA-binding domain-containing protein 5 Acyl-coenzyme A diphosphatase NUDT19 Peroxisomal coenzyme A diphosphatase NUDT7	A8MXV4 P0C024 Q5T8D3 Q8NC06 Q9NYQ3	ACBD4 ACBD5 HAO2 HAOX2 KIAA1996 NUDT19 NUDT7
Golgi Associated Vesicle Biogenesis	ADP-ribosylation factor 1 AP-1 complex subunit beta-1 AP-1 complex subunit gamma-1 AP-1 complex subunit mu-1 AP-1 complex subunit mu-2 AP-1 complex subunit sigma-1A AP-1 complex subunit sigma-2 AP-1 complex subunit sigma-3 AP-3 complex subunit beta-1 AP-3 complex subunit sigma-1 AP-4 complex subunit beta-1 AP-4 complex subunit epsilon-1 Adaptin ear-binding coat-associated protein 1 Alpha-soluble NSF attachment protein Auxilin Beta-arrestin-1 Biogenesis of lysosome-related organelles complex 1 subunit 1 Biogenesis of lysosome-related organelles complex 1 subunit 3 Biogenesis of lysosome-related organelles complex 1 subunit 4 Biogenesis of lysosome-related organelles complex 1 subunit 6 Carboxypeptidase D Cation-independent mannose-6-phosphate receptor Clathrin interactor 1 Cyclin-G-associated kinase Dynamin-2 Dysbindin Endophilin-A1 Ferritin heavy chain Ferritin light chain Golgi resident protein GCP60 Golgin subfamily B member 1 Heat shock cognate 71 kDa protein Huntingtin-interacting protein 1-related protein Inositol polyphosphate 5-phosphatase OCRL Phosphatidylinositol 4-phosphate 3-kinase C2 domain-containing subunit alpha Phosphatidylinositol-binding clathrin assembly protein Protein YIPF6 Pumilio homolog 1 Ras-related protein Rab-5C SH3 domain-containing protein 19 SNARE-associated protein Snapin Sortilin Sorting nexin-2 Sorting nexin-5 Sorting nexin-9 TBC1 domain family member 8B Thioredoxin domain-containing protein 5 Trans-Golgi network integral membrane protein 2 Transferrin receptor protein 1 Tumor protein D52 Tumor protein D53 Vesicle-associated membrane protein 2 Vesicle-associated membrane protein 7 Vesicle-associated membrane protein 8	O00203 O00443 O14976 O43493 O43747 O60749 O75061 O75146 O75976 O95295 P02786 P02792 P02794 P11142 P11717 P49407 P50570 P51148 P51809 P54920 P55327 P56377 P61966 P63027 P78537 P84077 Q01968 Q0IIM8 Q10567 Q13492 Q14671 Q14677 Q14789 Q16890 Q5HYK7 Q6QNY0 Q8NBS9 Q8NC96 Q92572 Q96EC8 Q96EV8 Q96PC3 Q99523 Q99962 Q9BV40 Q9BXS5 Q9H3P7 Q9NUP1 Q9UL45 Q9UPM8 Q9Y5X1 Q9Y5X3 Q9Y6B7 Q9Y6Q5	ACBD3 ADTB1 ADTB3A ADTG AP19 AP1B1 AP1G1 AP1M1 AP1M2 AP1S1 AP1S2 AP1S3 AP3B1 AP3S1 AP4B1 AP4E1 ARF1 ARR1 ARRB1 BAM22 BLOC1S1 BLOC1S3 BLOC1S4 BLOC1S6 BLOC1S7 BLOC1S8 BLOS1 BLOS3 CALM CLAPB2 CLAPG1 CLAPS1 CLAPS3 CLINT1 CLTNM CNO CNSA2 CPD DNAJC26 DNAJC6 DNM2 DTNBP1 DYN2 ENTH EPN4 EPNR FTH FTH1 FTHL6 FTL GAK GCN5L1 GCP60 GOCAP1 GOLGB1 GOLPH1 HIP12 HIP1R HSC70 HSP73 HSPA10 HSPA8 IGF2R KIAA0099 KIAA0171 KIAA0473 KIAA0655 MPRI NAPA NECAP1 OCRL OCRL1 PA PICALM PIK3C2A PLDN PUM1 PUMH1 RAB5C RABL RT14 SH3D19 SH3D2A SH3GL2 SH3PX1 SH3PXD3A SNAP25BP SNAPA SNAPAP SNAPIN SNX2 SNX5 SNX9 SORT1 SYB2 SYBL1 TBC1D8B TFRC TGN46 TGN51 TGOLN2 TLP46 TPD52 TPD52L1 TXNDC5 VAMP2 VAMP7 VAMP8 YIPF6
Defective SLC33A1 causes spastic paraplegia 42 (SPG42)	Acetyl-coenzyme A transporter 1	O00400	ACATN AT1 SLC33A1
Transport of vitamins, nucleosides, and related molecules	Acetyl-coenzyme A transporter 1 PDZ domain-containing protein 11 Sodium-dependent multivitamin transporter	O00400 Q5EBL8 Q9Y289	ACATN AIPP1 AT1 PDZD11 PDZK11 PISP SLC33A1 SLC5A6 SMVT
Ketone body catabolism	Acetyl-CoA acetyltransferase, mitochondrial D-beta-hydroxybutyrate dehydrogenase, mitochondrial Succinyl-CoA Succinyl-CoA	P24752 P55809 Q02338 Q9BYC2	ACAT ACAT1 BDH BDH1 MAT OXCT OXCT1 OXCT2 SCOT SDR9C1
Maturation of TCA enzymes and regulation of TCA cycle	Acetyl-CoA acetyltransferase, mitochondrial Aconitate hydratase, mitochondrial Citrate synthase, mitochondrial Citrate synthase-lysine N-methyltransferase CSKMT, mitochondrial Frataxin, mitochondrial Iron-sulfur cluster assembly 1 homolog, mitochondrial Iron-sulfur cluster assembly 2 homolog, mitochondrial Isocitrate dehydrogenase [NADP], mitochondrial LYR motif-containing protein 4 NAD-dependent protein deacetylase sirtuin-3, mitochondrial Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial Succinate dehydrogenase assembly factor 1, mitochondrial Succinate dehydrogenase assembly factor 2, mitochondrial Succinate dehydrogenase assembly factor 3, mitochondrial Succinate dehydrogenase assembly factor 4, mitochondrial Succinate dehydrogenase cytochrome b560 subunit, mitochondrial	A6NFY7 A8MUP2 O14521 O75390 P21912 P24752 P31040 P48735 Q16595 Q5VUM1 Q86U28 Q99643 Q99798 Q9BUE6 Q9HD34 Q9NRP4 Q9NTG7 Q9NX18	ACAT ACAT1 ACN9 ACO2 C11orf79 C6orf149 C6orf57 CS CSKMT CYB560 FRDA FXN HBLD1 HBLD2 IDH2 ISCA1 ISCA2 ISD11 LYRM4 LYRM8 MAT METTL12 PGL2 SDH SDH1 SDH2 SDH3 SDH4 SDH5 SDHA SDHAF1 SDHAF2 SDHAF3 SDHAF4 SDHB SDHC SDHD SDHF SIR2L3 SIRT3 X25
Transcriptional activation of mitochondrial biogenesis	3'-5' exoribonuclease HELZ2 5-aminolevulinate synthase, non-specific, mitochondrial ATP synthase subunit beta, mitochondrial Acetyl-coenzyme A synthetase, cytoplasmic CREB-binding protein CREB-regulated transcription coactivator 1 CREB-regulated transcription coactivator 2 CREB-regulated transcription coactivator 3 Calcium/calmodulin-dependent protein kinase type IV Calmodulin-1 Chromodomain-helicase-DNA-binding protein 9 Cyclic AMP-dependent transcription factor ATF-2 Cytochrome c DNA polymerase subunit gamma-2 DNA-directed RNA polymerase, mitochondrial Dimethyladenosine transferase 1, mitochondrial Dimethyladenosine transferase 2, mitochondrial F-box-like/WD repeat-containing protein TBL1X F-box-like/WD repeat-containing protein TBL1XR1 GA-binding protein alpha chain Glutamate dehydrogenase 1, mitochondrial Glutamate dehydrogenase 2, mitochondrial Histone deacetylase 3 Histone-arginine methyltransferase CARM1 Host cell factor 1 Isocitrate dehydrogenase [NADP], mitochondrial Mediator of RNA polymerase II transcription subunit 1 Myocyte-specific enhancer factor 2C Myocyte-specific enhancer factor 2D NAD-dependent protein deacetylase sirtuin-3, mitochondrial NAD-dependent protein deacylase sirtuin-5, mitochondrial NAD-dependent protein lipoamidase sirtuin-4, mitochondrial Nuclear receptor coactivator 1 Nuclear receptor coactivator 2 Nuclear receptor coactivator 6 Nuclear receptor corepressor 1 Nuclear receptor subfamily 1 group D member 1 Nuclear respiratory factor 1 PGC-1 and ERR-induced regulator in muscle protein 1 Peroxisome proliferator-activated receptor alpha Peroxisome proliferator-activated receptor gamma coactivator 1-alpha Peroxisome proliferator-activated receptor gamma coactivator 1-beta Peroxisome proliferator-activated receptor gamma coactivator-related protein 1 Retinoic acid receptor RXR-alpha SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily D member 3 Single-stranded DNA-binding protein, mitochondrial Steroid hormone receptor ERR1 Superoxide dismutase [Mn], mitochondrial Transcription factor A, mitochondrial Transcription termination factor 1, mitochondrial Trimethylguanosine synthase Twinkle mtDNA helicase	O00411 O15379 O60907 O75376 P00367 P04179 P06576 P0DP23 P11474 P13196 P15336 P19793 P20393 P48735 P49448 P51610 P99999 Q00059 Q04837 Q06413 Q06546 Q07869 Q14686 Q14814 Q15596 Q15648 Q15788 Q16566 Q16656 Q3L8U1 Q53ET0 Q5SV97 Q5VV67 Q6STE5 Q6UUV7 Q6UUV9 Q86X55 Q86YN6 Q8WVM0 Q92793 Q96RR1 Q96RS0 Q99551 Q9BYK8 Q9BZK7 Q9H5Q4 Q9NR19 Q9NTG7 Q9NXA8 Q9UBK2 Q9UHN1 Q9Y6E7	ACAS2 ACSS2 AIB3 ALAS1 ALAS3 ALASH ARC205 ATF2 ATP5B ATP5F1B ATPMB ATPSB BAF60C BHLHE74 BHLHE75 C10orf2 C1orf170 CALM CALM1 CAM CAM1 CAMK CAMK-GR CAMK4 CAMKIV CARM1 CBP CHD9 CREB2 CREBBP CREBP1 CRSP1 CRSP200 CRTC1 CRTC2 CRTC3 CYC CYCS DRIP205 DRIP230 E4TF1A EAR1 ERR1 ESRL1 ESRRA GABPA GLUD GLUD1 GLUD2 GLUDP1 HCA137 HCF1 HCFC1 HDAC3 HELZ2 HFC1 HREV IDH2 IRA1 KIAA0181 KIAA0308 KIAA0595 KIAA0616 KIAA1047 KIAA1769 KISH2 LEM6 MECT1 MED1 MEF2C MEF2D MTERF MTERF1 MTPOLB NCOA1 NCOA2 NCOA6 NCOA6IP NCOR1 NR1C1 NR1D1 NR2B1 NR3B1 NRF1 NS5ATP5 PBP PEO1 PERC PERM1 PGC1 PGC1A PGC1B PIMT POLG2 POLRMT PPAR PPARA PPARBP PPARGBP PPARGC1 PPARGC1A PPARGC1B PPRC1 PRIC285 PRIC320 PRMT4 RAP250 RB18A RXRA SIR2L3 SIR2L4 SIR2L5 SIRT3 SIRT4 SIRT5 SMARCD3 SOD2 SRC1 SRC2 SSBP SSBP1 TBL1 TBL1X TBL1XR1 TBLR1 TCF6 TCF6L2 TFAM TFB1M TFB2M TGS1 THRAL TIF2 TORC1 TORC2 TORC3 TRAP220 TRBP TRIP2 TWNK WAMTP1
Ethanol oxidation	Acetyl-coenzyme A synthetase 2-like, mitochondrial Acetyl-coenzyme A synthetase, cytoplasmic Alcohol dehydrogenase 1A Alcohol dehydrogenase 1C Alcohol dehydrogenase 6 Alcohol dehydrogenase class-3 Aldehyde dehydrogenase 1A1 Aldehyde dehydrogenase X, mitochondrial Aldehyde dehydrogenase, mitochondrial All-trans-retinol dehydrogenase [NAD(+)] ADH1B All-trans-retinol dehydrogenase [NAD(+)] ADH4 All-trans-retinol dehydrogenase [NAD(+)] ADH7	P00325 P00326 P00352 P05091 P07327 P08319 P11766 P28332 P30837 P40394 Q9NR19 Q9NUB1	ACAS2 ACAS2L ACSS1 ACSS2 ADH1 ADH1A ADH1B ADH1C ADH2 ADH3 ADH4 ADH5 ADH6 ADH7 ADHX ALDC ALDH1 ALDH1A1 ALDH1B1 ALDH2 ALDH5 ALDHX ALDM FDH KIAA1846 PUMB1
Defective ST3GAL3 causes MCT12 and EIEE15	Aggrecan core protein CMP-N-acetylneuraminate-beta-1,4-galactoside alpha-2,3-sialyltransferase Fibromodulin Keratocan Lumican Mimecan Osteomodulin Prolargin	O60938 P16112 P20774 P51884 P51888 Q06828 Q11203 Q99983	ACAN AGC1 CSPG1 FM FMOD KERA LDC LUM MSK16 OGN OIF OMD PRELP SIAT6 SLRR2A SLRR2B SLRR2C SLRR2D SLRR2E SLRR3A ST3GAL3
Keratan sulfate degradation	Aggrecan core protein Beta-galactosidase-1-like protein 2 Beta-galactosidase-1-like protein 3 Beta-galactosidase-1-like protein Beta-galactosidase Beta-hexosaminidase subunit alpha Beta-hexosaminidase subunit beta Fibromodulin Keratocan Lumican Mimecan N-acetylglucosamine-6-sulfatase Osteomodulin Prolargin	O60938 P06865 P07686 P15586 P16112 P16278 P20774 P51884 P51888 Q06828 Q6UWU2 Q8IW92 Q8NCI6 Q99983	ACAN AGC1 CSPG1 ELNR1 FM FMOD GLB1 GLB1L GLB1L2 GLB1L3 GNS HEXA HEXB KERA LDC LUM MSK16 OGN OIF OMD PRELP SLRR2A SLRR2B SLRR2C SLRR2D SLRR2E SLRR3A	G09546QO G20239CJ G27025MB G50451AA G80016QC G82768YB G85384LV
Defective CHST6 causes MCDC1	Aggrecan core protein Carbohydrate sulfotransferase 6 Fibromodulin Keratocan Lumican Mimecan Osteomodulin Prolargin	O60938 P16112 P20774 P51884 P51888 Q06828 Q99983 Q9GZX3	ACAN AGC1 CHST6 CSPG1 FM FMOD KERA LDC LUM MSK16 OGN OIF OMD PRELP SLRR2A SLRR2B SLRR2C SLRR2D SLRR2E SLRR3A
Defective B4GALT1 causes B4GALT1-CDG (CDG-2d)	Aggrecan core protein Beta-1,4-galactosyltransferase 1 Fibromodulin Keratocan Lumican Mimecan Osteomodulin Prolargin	O60938 P15291 P16112 P20774 P51884 P51888 Q06828 Q99983	ACAN AGC1 B4GALT1 CSPG1 FM FMOD GGTB2 KERA LDC LUM MSK16 OGN OIF OMD PRELP SLRR2A SLRR2B SLRR2C SLRR2D SLRR2E SLRR3A
Keratan sulfate biosynthesis	Aggrecan core protein Beta-1,4-galactosyltransferase 1 Beta-1,4-galactosyltransferase 2 Beta-1,4-galactosyltransferase 3 Beta-1,4-galactosyltransferase 4 Beta-1,4-galactosyltransferase 5 Beta-1,4-galactosyltransferase 6 Beta-1,4-glucuronyltransferase 1 CMP-N-acetylneuraminate-beta-1,4-galactoside alpha-2,3-sialyltransferase CMP-N-acetylneuraminate-beta-galactosamide-alpha-2,3-sialyltransferase 1 CMP-N-acetylneuraminate-beta-galactosamide-alpha-2,3-sialyltransferase 2 CMP-N-acetylneuraminate-beta-galactosamide-alpha-2,3-sialyltransferase 4 Carbohydrate sulfotransferase 1 Carbohydrate sulfotransferase 2 Carbohydrate sulfotransferase 5 Carbohydrate sulfotransferase 6 Fibromodulin Keratocan Lumican Mimecan N-acetyllactosaminide beta-1,3-N-acetylglucosaminyltransferase 2 N-acetyllactosaminide beta-1,3-N-acetylglucosaminyltransferase 3 N-acetyllactosaminide beta-1,3-N-acetylglucosaminyltransferase 4 Nucleotide sugar transporter SLC35D2 Osteomodulin Prolargin Type 2 lactosamine alpha-2,3-sialyltransferase UDP-GlcNAc	O43286 O43505 O43916 O60512 O60513 O60909 O60938 P15291 P16112 P20774 P51884 P51888 Q06828 Q11201 Q11203 Q11206 Q16842 Q76EJ3 Q8NFL0 Q99983 Q9C0J1 Q9GZS9 Q9GZX3 Q9NY97 Q9UBX8 Q9Y274 Q9Y2A9 Q9Y4C5	ACAN AGC1 B3GALT7 B3GALT8 B3GNT1 B3GNT2 B3GNT3 B3GNT4 B3GNT6 B3GNT7 B4GALT1 B4GALT2 B4GALT3 B4GALT4 B4GALT5 B4GALT6 B4GAT1 CGS23 CHST1 CHST2 CHST5 CHST6 CSPG1 FM FMOD GGTB2 GN6ST HFRC KERA LDC LUM MSK16 NANTA3 OGN OIF OMD PRELP SIAT10 SIAT4 SIAT4A SIAT4B SIAT4C SIAT6 SLC35D2 SLRR2A SLRR2B SLRR2C SLRR2D SLRR2E SLRR3A ST3GAL1 ST3GAL2 ST3GAL3 ST3GAL4 ST3GAL6 STZ TMEM3 UGTREL8
ECM proteoglycans	Aggrecan core protein Agrin Asporin Basement membrane-specific heparan sulfate proteoglycan core protein Biglycan Brevican core protein Cartilage matrix protein Cartilage oligomeric matrix protein Collagen alpha-1(IX) chain Collagen alpha-2(IX) chain Collagen alpha-3(IX) chain Decorin Dentin matrix acidic phosphoprotein 1 Dentin sialophosphoprotein Dystroglycan 1 Fibromodulin Hyaluronan and proteoglycan link protein 1 Integrin alpha-2 Integrin alpha-7 Integrin alpha-8 Integrin alpha-9 Integrin alpha-IIb Integrin alpha-V Integrin alpha-X Integrin beta-1 Integrin beta-3 Integrin beta-5 Integrin beta-6 Integrin-binding sialoprotein Laminin subunit alpha-1 Laminin subunit alpha-2 Laminin subunit alpha-3 Laminin subunit alpha-4 Laminin subunit alpha-5 Laminin subunit beta-1 Laminin subunit beta-2 Laminin subunit gamma-1 Low-density lipoprotein receptor-related protein 4 Lumican Matrilin-3 Matrilin-4 Muscle, skeletal receptor tyrosine-protein kinase Neural cell adhesion molecule 1 Neurocan core protein Plasminogen activator inhibitor 1 Receptor-type tyrosine-protein phosphatase S SPARC Tenascin-N Tenascin-R Tenascin-X Tenascin Transforming growth factor beta-1 proprotein Transforming growth factor beta-2 proprotein Transforming growth factor beta-3 proprotein Versican core protein Vitronectin	O00468 O14594 O15146 O15230 O15232 O75096 O95460 P01137 P04004 P05106 P05121 P05556 P06756 P07585 P07942 P08514 P09486 P10600 P10915 P11047 P13591 P13611 P16112 P17301 P18084 P18564 P20702 P20849 P21810 P21815 P21941 P22105 P24043 P24821 P25391 P49747 P51884 P53708 P55268 P61812 P98160 Q06828 Q13316 Q13332 Q13683 Q13797 Q14050 Q14055 Q14118 Q16363 Q16787 Q92752 Q96GW7 Q9BXN1 Q9NZW4 Q9UQP3	ACAN AGC1 AGRIN AGRN ASPN BCAN BEHAB BGN BNSP CD11C CD49B CMP COL9A1 COL9A2 COL9A3 COMP CRTL1 CRTM CSPG1 CSPG2 CSPG3 CSPG7 DAG1 DCN DMP1 DSPP FM FMOD FNRB GP2B GP3A HAPLN1 HSPG2 HXB HXBL IBSP ITGA2 ITGA2B ITGA7 ITGA8 ITGA9 ITGAB ITGAV ITGAX ITGB1 ITGB3 ITGB5 ITGB6 KIAA0533 KIAA0816 KIAA1907 LAMA LAMA1 LAMA2 LAMA3 LAMA4 LAMA5 LAMB1 LAMB2 LAMC1 LAMM LAMNA LAMS LDC LRP10 LRP4 LUM MATN1 MATN3 MATN4 MDF2 MEGF7 MSK12 MSK16 MSK8 MUSK NCAM NCAM1 NCAN NEUR ON PAI1 PLANH1 PLAP1 PTPRS SERPINE1 SLRR1A SLRR1B SLRR1C SLRR2D SLRR2E SPARC TGFB TGFB1 TGFB2 TGFB3 TNC TNN TNR TNW TNX TNXB TNXB1 TNXB2 VCAN VNRA VTN VTNR XB
Beta oxidation of palmitoyl-CoA to myristoyl-CoA	Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Very long-chain specific acyl-CoA dehydrogenase, mitochondrial	P40939 P49748 P55084	ACADVL HADH HADHA HADHB VLCAD
Beta oxidation of hexanoyl-CoA to butanoyl-CoA	Enoyl-CoA hydratase, mitochondrial Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial Short-chain specific acyl-CoA dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial	P16219 P30084 P40939 P55084 Q16836	ACADS ECHS1 HAD HAD1 HADH HADHA HADHB HADHSC SCHAD
Beta oxidation of butanoyl-CoA to acetyl-CoA	Acyl-coenzyme A synthetase ACSM3, mitochondrial Acyl-coenzyme A synthetase ACSM6, mitochondrial Enoyl-CoA hydratase, mitochondrial Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial Short-chain specific acyl-CoA dehydrogenase, mitochondrial	P16219 P30084 Q16836 Q53FZ2 Q6P461	ACADS ACSM3 ACSM6 C10orf129 ECHS1 HAD HAD1 HADH HADHSC SAH SCHAD
Beta oxidation of octanoyl-CoA to hexanoyl-CoA	Enoyl-CoA hydratase, mitochondrial Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial	P11310 P30084 P40939 P55084 Q16836	ACADM ECHS1 HAD HAD1 HADH HADHA HADHB HADHSC SCHAD
Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA	Enoyl-CoA hydratase, mitochondrial Enoyl-[acyl-carrier-protein] reductase, mitochondrial Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial	P11310 P30084 P40939 P55084 Q16836 Q9BV79	ACADM ECHS1 HAD HAD1 HADH HADHA HADHB HADHSC MECR NBRF1 SCHAD
Beta oxidation of myristoyl-CoA to lauroyl-CoA	Long-chain specific acyl-CoA dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial	P28330 P40939 P55084	ACADL HADH HADHA HADHB
Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA	Enoyl-CoA hydratase, mitochondrial Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial	P28330 P30084 P40939 P55084 Q16836	ACADL ECHS1 HAD HAD1 HADH HADHA HADHB HADHSC SCHAD
mitochondrial fatty acid beta-oxidation of unsaturated fatty acids	2,4-dienoyl-CoA reductase [(3E)-enoyl-CoA-producing], mitochondrial Enoyl-CoA delta isomerase 1, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial	P11310 P28330 P40939 P42126 P55084 Q16698	ACADL ACADM DCI DECR DECR1 ECI1 HADH HADHA HADHB SDR18C1
Complex I biogenesis	Acyl carrier protein, mitochondrial Arginine-hydroxylase NDUFAF5, mitochondrial Complex I assembly factor ACAD9, mitochondrial Complex I assembly factor TIMMDC1, mitochondrial Complex I assembly factor TMEM126B, mitochondrial Complex I intermediate-associated protein 30, mitochondrial Cytochrome c oxidase assembly factor 1 homolog Evolutionarily conserved signaling intermediate in Toll pathway, mitochondrial Iron-sulfur cluster transfer protein NUBPL NADH dehydrogenase (ubiquinone) complex I, assembly factor 6 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial NADH dehydrogenase [ubiquinone] 1 subunit C2 NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial NADH-ubiquinone oxidoreductase chain 1 NADH-ubiquinone oxidoreductase chain 2 NADH-ubiquinone oxidoreductase chain 3 NADH-ubiquinone oxidoreductase chain 4 NADH-ubiquinone oxidoreductase chain 5 NADH-ubiquinone oxidoreductase chain 6 Protein arginine methyltransferase NDUFAF7, mitochondrial Transmembrane protein 186	O00217 O14561 O15239 O43181 O43674 O43676 O43677 O43678 O43920 O75251 O75306 O75380 O75438 O75489 O95139 O95167 O95168 O95169 O95178 O95182 O95298 O95299 O96000 P03886 P03891 P03897 P03905 P03915 P03923 P17568 P19404 P28331 P49821 P51970 P56181 P56556 Q16718 Q16795 Q330K2 Q5TEU4 Q7L592 Q86Y39 Q8IUX1 Q8N183 Q8TB37 Q96B77 Q9BQ95 Q9BU61 Q9GZY4 Q9H845 Q9NPL8 Q9NX14 Q9P032 Q9P0J0 Q9UI09 Q9Y375 Q9Y6M9	ACAD9 C14orf127 C16orf51 C20orf7 C2orf56 C3orf1 C3orf60 C6orf66 C7orf44 C8orf38 CIA30 COA1 DAP13 ECSIT GRIM19 HRPAP20 LYRM3 LYRM6 MITRAC15 MT-ND1 MT-ND2 MT-ND3 MT-ND4 MT-ND5 MT-ND6 MTND1 MTND2 MTND3 MTND4 MTND5 MTND6 NADH1 NADH2 NADH3 NADH4 NADH5 NADH6 NADHB14 ND1 ND2 ND3 ND4 ND5 ND6 NDUFA1 NDUFA10 NDUFA11 NDUFA12 NDUFA12L NDUFA13 NDUFA2 NDUFA3 NDUFA5 NDUFA6 NDUFA7 NDUFA8 NDUFA9 NDUFAB1 NDUFAF1 NDUFAF2 NDUFAF3 NDUFAF4 NDUFAF5 NDUFAF6 NDUFAF7 NDUFB1 NDUFB10 NDUFB11 NDUFB2 NDUFB3 NDUFB4 NDUFB5 NDUFB6 NDUFB7 NDUFB8 NDUFB9 NDUFC1 NDUFC2 NDUFS1 NDUFS2 NDUFS2L NDUFS3 NDUFS4 NDUFS5 NDUFS6 NDUFS7 NDUFS8 NDUFV1 NDUFV2 NDUFV3 NUBPL TIMMDC1 TMEM126B TMEM186 UQOR1 UQOR22
Branched-chain amino acid catabolism	2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial 3-hydroxyacyl-CoA dehydrogenase type-2 3-hydroxyisobutyrate dehydrogenase, mitochondrial 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial Branched-chain alpha-ketoacid dehydrogenase kinase Branched-chain-amino-acid aminotransferase, cytosolic Branched-chain-amino-acid aminotransferase, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial Isobutyryl-CoA dehydrogenase, mitochondrial Isovaleryl-CoA dehydrogenase, mitochondrial Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial Methylglutaconyl-CoA hydratase, mitochondrial Methylmalonate-semialdehyde/malonate-semialdehyde dehydrogenase [acylating], mitochondrial Protein phosphatase Mn(2+)-dependent 1K Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial Solute carrier family 25 member 44	O14874 O15382 P09622 P11182 P12694 P21953 P24752 P26440 P30084 P31937 P45954 P54687 Q02252 Q13825 Q6NVY1 Q8N3J5 Q96H78 Q96RQ3 Q99714 Q9HCC0 Q9UKU7	ACAD8 ACADSB ACAT ACAT1 ALDH6A1 ARC42 AUH BCAT1 BCAT2 BCATE2 BCATM BCKDHA BCKDHB BCKDHE2 BCKDK BCT1 BCT2 DBT DLD ECA39 ECA40 ECHS1 ERAB GCSL HADH2 HIBADH HIBCH HSD17B10 IBD IVD KIAA0446 LAD MAT MCCA MCCB MCCC1 MCCC2 MMSDH MRPP2 PHE3 PP2CM PPM1K SCHAD SDR5C1 SLC25A44 XH98G2

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