Reviewed, UniProtKB/Swiss-Prot Q10713 (MPPA_HUMAN)

Names and origin

Protein names

Recommended name:
    Mitochondrial-processing peptidase subunit alpha
    EC=3.4.24.64
Alternative name(s):
    Alpha-MPP
    P-55

Gene names

Name:	PMPCA
Synonyms:	INPP5E, KIAA0123, MPPA

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

Protein attributes

Sequence length	525 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level.

General annotation (Comments)

Function	Cleaves presequences (transit peptides) from mitochondrial protein precursors By similarity.
Catalytic activity	Release of N-terminal transit peptides from precursor proteins imported into the mitochondrion, typically with Arg in position P2.
Subunit structure	Heterodimer of alpha and beta subunits By similarity.
Subcellular location	Mitochondrion matrix By similarity.
Sequence similarities	Belongs to the peptidase M16 family.
Caution	Does not seem to have a protease activity as it lack the zinc-binding site.
Mass spectrometry	Molecular mass is 54624.57 Da from positions 33 - 525. Determined by MALDI. Ref.4

Ontologies

Keywords
Cellular component	Mitochondrion
Domain	Transit peptide
Molecular function	Hydrolase Metalloprotease Protease
Gene Ontology (GO)
Biological process	proteolysis Traceable author statement. Source: UniProtKB
Cellular component	mitochondrial inner membrane Traceable author statement. Source: UniProtKB mitochondrial matrix Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular function	metalloendopeptidase activity Traceable author statement. Source: UniProtKB protein binding Inferred from direct assay. Source: UniProtKB zinc ion binding Inferred from electronic annotation. Source: InterPro
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 33	33	Mitochondrion
	Chain	34 – 525	492	Mitochondrial-processing peptidase subunit alpha		PRO_0000026767
Experimental info
	Sequence conflict	495	1	G → C in AAH22949. Ref.3
	Sequence conflict	505	1	H → D in BAA04643. Ref.1

Sequences

	Sequence		Length	Mass (Da)
	Q10713-1 [UniParc]. Last modified April 11, 2003. Version 2. Checksum: 2751E7FCDC864E3F	FASTA	525	58,253
	10 20 30 40 50 60 MAAVVLAATR LLRGSGSWGC SRLRFGPPAY RRFSSGGAYP NIPLSSPLPG VPKPVFATVD 70 80 90 100 110 120 GQEKFETKVT TLDNGLRVAS QNKFGQFCTV GILINSGSRY EAKYLSGIAH FLEKLAFSST 130 140 150 160 170 180 ARFDSKDEIL LTLEKHGGIC DCQTSRDTTM YAVSADSKGL DTVVALLADV VLQPRLTDEE 190 200 210 220 230 240 VEMTRMAVQF ELEDLNLRPD PEPLLTEMIH EAAYRENTVG LHRFCPTENV AKINREVLHS 250 260 270 280 290 300 YLRNYYTPDR MVLAGVGVEH EHLVDCARKY LLGVQPAWGS AEAVDIDRSV AQYTGGIAKL 310 320 330 340 350 360 ERDMSNVSLG PTPIPELTHI MVGLESCSFL EEDFIPFAVL NMMMGGGGSF SAGGPGKGMF 370 380 390 400 410 420 SRLYLNVLNR HHWMYNATSY HHSYEDTGLL CIHASADPRQ VREMVEIITK EFILMGGTVD 430 440 450 460 470 480 TVELERAKTQ LTSMLMMNLE SRPVIFEDVG RQVLATRSRK LPHELCTLIR NVKPEDVKRV 490 500 510 520 ASKMLRGKPA VAALGDLTDL PTYEHIQTAL SSKDGRLPRT YRLFR

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Prediction of the coding sequences of unidentified human genes. III. The coding sequences of 40 new genes (KIAA0081-KIAA0120) deduced by analysis of cDNA clones from human cell line KG-1." Nagase T., Miyajima N., Tanaka A., Sazuka T., Seki N., Sato S., Tabata S., Ishikawa K., Kawarabayasi Y., Kotani H., Nomura N. DNA Res. 2:37-43(1995) [PubMed: 7788527] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Bone marrow.
[2]	"Prediction of the coding sequences of unidentified human genes. IV. The coding sequences of 40 new genes (KIAA0121-KIAA0160) deduced by analysis of cDNA clones from human cell line KG-1." Nagase T., Seki N., Tanaka A., Ishikawa K., Nomura N. DNA Res. 2:167-174(1995) [PubMed: 8590280] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Bone marrow.
[3]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Lymph and Ovary.
[4]	"Cluster analysis of an extensive human breast cancer cell line protein expression map database." Harris R.A., Yang A., Stein R.C., Lucy K., Brusten L., Herath A., Parekh R., Waterfield M.D., O'Hare M.J., Neville M.A., Page M.J., Zvelebil M.J. Proteomics 2:212-223(2002) [PubMed: 11840567] [Abstract] Cited for: MASS SPECTROMETRY. Tissue: Mammary cancer.
[5]	Colinge J., Superti-Furga G., Bennett K.L. Submitted (OCT-2008) to UniProtKB Cited for: IDENTIFICATION [LARGE SCALE ANALYSIS], MASS SPECTROMETRY.

Cross-references

Sequence databases
EMBLGenBankDDBJ	D21064 mRNA. Translation: BAA04643.1. Different initiation. D50913 mRNA. Translation: BAA09472.2. Different initiation. BC022949 mRNA. Translation: AAH22949.1. BC033103 mRNA. Translation: AAH33103.2. Different initiation.
IPI	IPI00166749.
RefSeq	NP_055975.1.
UniGene	Hs.495471
3D structure databases
HSSP	HSSP built from PDB template 1HR6 based on UniProtKB P11914.
ModBase	Search...
Protein family/group databases
MEROPS	M16.971.
PTM databases
PhosphoSite	Q10713.
Proteomic databases
PeptideAtlas	Q10713.
PRIDE	Q10713.
Genome annotation databases
Ensembl	ENSG00000165688. Homo sapiens. [Contig view]
GeneID	23203.
KEGG	hsa:23203.
Organism-specific databases
GeneCards	GC09P138424.
H-InvDB	HIX0008545.
HGNC	HGNC:18667. PMPCA.
PharmGKB	PA38629.
HUGE	Search...
GenAtlas	Search...
Phylogenomic databases
HOGENOM	Q10713.
HOVERGEN	Q10713.
OMA	Q10713. YRGNTVG.
Enzyme and pathway databases
BRENDA	3.4.24.64. 247.
Gene expression databases
ArrayExpress	Q10713.
Bgee	Q10713.
CleanEx	HS_INPP5E. HS_PMPCA.
GermOnline	ENSG00000165688. Homo sapiens.
Family and domain databases
InterPro	IPR011237. Pept_M16_core. IPR011765. Pept_M16_N. IPR001431. Pept_M16_Zn_BS. IPR007863. Peptidase_M16_C. [Graphical view]
Gene3D	G3DSA:3.30.830.10. Pept_M16_core. 2 hits.
Pfam	PF00675. Peptidase_M16. 1 hit. PF05193. Peptidase_M16_C. 1 hit. [Graphical view]
PROSITE	PS00143. INSULINASE. 1 hit. [Graphical view]
ProtoNet	Search...
Other Resources
NextBio	44723.

Entry information

Entry name

MPPA_HUMAN

Accession

Primary (citable) accession number: Q10713
Secondary accession number(s): Q16639, Q8N513

Entry history

Integrated into UniProtKB/Swiss-Prot:	October 1, 1996
Last sequence update:	April 11, 2003
Last modified:	June 16, 2009
This is version 91 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)