Q10713 (MPPA_HUMAN) Reviewed, UniProtKB/Swiss-Prot

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
Sequence caution	The sequence AAH33103.2 differs from that shown. Reason: Erroneous initiation. The sequence BAA04643.1 differs from that shown. Reason: Erroneous initiation. The sequence BAA09472.2 differs from that shown. Reason: Erroneous initiation.

Ontologies

Keywords
Cellular component	Mitochondrion
Domain	Transit peptide
Molecular function	Hydrolase Metalloprotease Protease
PTM	Acetylation
Technical term	Complete proteome Reference proteome
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 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
Amino acid modifications
	Modified residue	299	1	N6-acetyllysine Ref.5
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 [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]	"Lysine acetylation targets protein complexes and co-regulates major cellular functions." Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T., Olsen J.V., Mann M. Science 325:834-840(2009) [PubMed: 19608861] [Abstract] Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-299, MASS SPECTROMETRY.
[6]	"Initial characterization of the human central proteome." Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J. BMC Syst. Biol. 5:17-17(2011) [PubMed: 21269460] [Abstract] Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	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. NM_015160.1.
UniGene	Hs.495471.
3D structure databases
ProteinModelPortal	Q10713.
SMR	Q10713. Positions 65-510.
ModBase	Search...
Protein-protein interaction databases
IntAct	Q10713. 1 interaction.
STRING	Q10713.
Protein family/group databases
MEROPS	M16.971.
PTM databases
PhosphoSite	Q10713.
Polymorphism databases
DMDM	29840846.
Proteomic databases
PeptideAtlas	Q10713.
PRIDE	Q10713.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000371717; ENSP00000360782; ENSG00000165688.
GeneID	23203.
KEGG	hsa:23203.
UCSC	uc004chl.1. human.
Organism-specific databases
CTD	23203.
GeneCards	GC09P139305.
H-InvDB	HIX0008545.
HGNC	HGNC:18667. PMPCA.
HPA	HPA021648.
MIM	613036. gene.
neXtProt	NX_Q10713.
PharmGKB	PA38629.
HUGE	Search...
GenAtlas	Search...
Phylogenomic databases
eggNOG	prNOG06716.
HOGENOM	HBG446511.
HOVERGEN	HBG106890.
InParanoid	Q10713.
OMA	EFILMAG.
PhylomeDB	Q10713.
Gene expression databases
ArrayExpress	Q10713.
Bgee	Q10713.
CleanEx	HS_INPP5E. HS_PMPCA.
Genevestigator	Q10713.
GermOnline	ENSG00000165688. Homo sapiens.
Family and domain databases
InterPro	IPR011249. Metalloenz_metal-bd. 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.
KO	K01412.
Pfam	PF00675. Peptidase_M16. 1 hit. PF05193. Peptidase_M16_C. 1 hit. [Graphical view]
SUPFAM	SSF63411. Metalloenz_metal-bd. 2 hits.
PROSITE	PS00143. INSULINASE. 1 hit. [Graphical view]
ProtoNet	Search...
Other
NextBio	44723.
SOURCE	Search...

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:	January 25, 2012
This is version 115 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.