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peptidase, mitochondrial processing subunit alpha

Summary

Gene Symbol

PMPCA

Organism

Homo sapiens (human)

External Links

NCBI Gene

23203

PubChem

23203

Alliance of Genome Resources

HGNC:18667

Annotation

Keyword

Acetylation
Alternative splicing
Disease variant
Intellectual disability
Mitochondrion inner membrane
Neurodegeneration
Proteomics identification
Reference proteome
Transit peptide

Proteins

Displaying **all 2** entries
UniProt	Protein Name
Q10713	Alpha-MPP Inactive zinc metalloprotease alpha P-55
Q5SXN9

Annotation information from UniProt.

Gene Ontology (GO)

Displaying **all 4** entries
GO Term	Evidence Code	PMID
protein processing involved in protein targeting to mitochondrion	NAS	32443488
protein processing involved in protein targeting to mitochondrion	IDA	22354088
protein processing involved in protein targeting to mitochondrion	IMP	25808372
protein processing involved in protein targeting to mitochondrion	IBA

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

organonitrogen compound metabolic process [inference]

protein metabolic process [inference]

proteolysis [inference]

protein processing [inference]

mitochondrial protein processing [inference]

protein processing involved in protein targeting to mitochondrion

protein maturation [inference]

protein processing [inference]

mitochondrial protein processing [inference]

protein processing involved in protein targeting to mitochondrion

primary metabolic process [inference]

protein metabolic process [inference]

proteolysis [inference]

protein processing [inference]

mitochondrial protein processing [inference]

protein processing involved in protein targeting to mitochondrion

protein maturation [inference]

protein processing [inference]

mitochondrial protein processing [inference]

protein processing involved in protein targeting to mitochondrion

organic substance metabolic process [inference]

macromolecule metabolic process [inference]

protein metabolic process [inference]

proteolysis [inference]

protein processing [inference]

mitochondrial protein processing [inference]

protein processing involved in protein targeting to mitochondrion

protein maturation [inference]

protein processing [inference]

mitochondrial protein processing [inference]

protein processing involved in protein targeting to mitochondrion

organonitrogen compound metabolic process [inference]

protein metabolic process [inference]

proteolysis [inference]

protein processing [inference]

mitochondrial protein processing [inference]

protein processing involved in protein targeting to mitochondrion

protein maturation [inference]

protein processing [inference]

mitochondrial protein processing [inference]

protein processing involved in protein targeting to mitochondrion

Displaying entries **1 - 5** of 8 in total

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GO Term	Evidence Code	PMID
extracellular space	HDA	22664934
mitochondrion	NAS	32443488
mitochondrion	IDA
mitochondrion	IBA
mitochondrion	HTP	34800366

GO Hierarchy

cellular component

protein-containing complex [inference]

mitochondrial protein-containing complex [inference]

mitochondrial processing peptidase complex

catalytic complex [inference]

mitochondrial processing peptidase complex

cellular anatomical entity [inference]

extracellular space

membrane [inference]

organelle membrane [inference]

organelle inner membrane [inference]

mitochondrial inner membrane

mitochondrial membrane [inference]

mitochondrial inner membrane

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

mitochondrial matrix

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

Displaying **all 3** entries
GO Term	Evidence Code	PMID
metalloendopeptidase activity	IEA
protein binding	IPI	30021884 32296183 33961781
metal ion binding	IEA

GO Hierarchy

molecular function

catalytic activity [inference]

hydrolase activity [inference]

peptidase activity [inference]

endopeptidase activity [inference]

metalloendopeptidase activity

metallopeptidase activity [inference]

metalloendopeptidase activity

catalytic activity, acting on a protein [inference]

peptidase activity [inference]

endopeptidase activity [inference]

metalloendopeptidase activity

metallopeptidase activity [inference]

metalloendopeptidase activity

binding [inference]

protein binding

ion binding [inference]

cation binding [inference]

metal ion binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

Peptidase

Functional Category

K: Transcription
M: Cell wall/membrane/envelope biogenesis
O: Posttranslational modification, protein turnover, chaperones

Displaying **all 4** entries
Gene Ontology
metal ion binding
metalloendopeptidase activity
protein processing involved in protein targeting to mitochondrion
proteolysis

Displaying **all 4** entries
InterPro
Metalloenzyme, LuxS/M16 peptidase-like
Peptidase M16, C-terminal
Peptidase M16, N-terminal
Peptidase M16, zinc-binding site

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:0080061	autosomal recessive spinocerebellar ataxia 2	Alliance of Genome Resources

Ortholog

Displaying entries **1 - 10** of 74 in total

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Xenopus tropicalis	23203	Xenbase:XB-GENE-977435
Mus musculus	66865	Xenbase:XB-GENE-977435	MOUSE35582
Rattus norvegicus	296588		RATNO27231
Canis lupus familiaris	480677		CANLF19267
Danio rerio	492801		DANRE36867
Macaca mulatta	721742		MACMU12956
Bos taurus	767847		BOVIN03416
Monodelphis domestica	100020320		MONDO05706
Equus caballus	100068710		HORSE23903
Ornithorhynchus anatinus	100092395		ORNAN21029

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OrthoDB (Group)

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Acknowledgements