Reviewed, UniProtKB/Swiss-Prot O75439 (MPPB_HUMAN)

Names and origin

Protein names

Recommended name:
    Mitochondrial-processing peptidase subunit beta
    EC=3.4.24.64
Alternative name(s):
    Beta-MPP
    P-52

Gene names

Name:	PMPCB
Synonyms:	MPPB

Organism

Homo sapiens (Human) [Complete proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

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

Protein attributes

Sequence length	489 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.
Cofactor	Binds 1 zinc ion per subunit By similarity.
Subunit structure	Heterodimer of alpha and beta subunits By similarity.
Subcellular location	Mitochondrion matrix By similarity.
Sequence similarities	Belongs to the peptidase M16 family.

Ontologies

Keywords
Cellular component	Mitochondrion
Coding sequence diversity	Polymorphism
Domain	Transit peptide
Ligand	Metal-binding Zinc
Molecular function	Hydrolase Metalloprotease Protease
Technical term	Complete proteome
Gene Ontology (GO)
Biological process	proteolysis Inferred from electronic annotation. Source: InterPro
Cellular component	mitochondrial matrix Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular function	metalloendopeptidase activity Inferred from electronic annotation. Source: InterPro zinc ion binding Inferred from electronic annotation. Source: UniProtKB-KW
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 45	45	Mitochondrion Potential
	Chain	46 – 489	444	Mitochondrial-processing peptidase subunit beta		PRO_0000026777
Sites
	Active site	104	1	Proton acceptor By similarity
	Metal binding	101	1	Zinc By similarity
	Metal binding	105	1	Zinc By similarity
	Metal binding	181	1	Zinc By similarity
Natural variations
	Natural variant	396	1	E → D: dbSNP rs3087615.		VAR_051572
Experimental info
	Sequence conflict	16 – 17	2	RR → GG in AAC39915. Ref.1
	Sequence conflict	470 – 489	20	PIKQL…CWLRD → KPGFFSSMQKVGQVLLINSS F in AAH10398. Ref.3

Sequences

	Sequence		Length	Mass (Da)
	O75439-1 [UniParc]. Last modified April 11, 2003. Version 2. Checksum: 79250D016E60CFEE	FASTA	489	54,366
	10 20 30 40 50 60 MAAAAARVVL SSAARRRLWG FSESLLIRGA AGRSLYFGEN RLRSTQAATQ VVLNVPETRV 70 80 90 100 110 120 TCLESGLRVA SEDSGLSTCT VGLWIDAGSR YENEKNNGTA HFLEHMAFKG TKKRSQLDLE 130 140 150 160 170 180 LEIENMGAHL NAYTSREQTV YYAKAFSKDL PRAVEILADI IQNSTLGEAE IERERGVILR 190 200 210 220 230 240 EMQEVETNLQ EVVFDYLHAT AYQNTALGRT ILGPTENIKS ISRKDLVDYI TTHYKGPRIV 250 260 270 280 290 300 LAAAGGVSHD ELLDLAKFHF GDSLCTHKGE IPALPPCKFT GSEIRVRDDK MPLAHLAIAV 310 320 330 340 350 360 EAVGWAHPDT ICLMVANTLI GNWDRSFGGG MNLSSKLAQL TCHGNLCHSF QSFNTSYTDT 370 380 390 400 410 420 GLWGLYMVCE SSTVADMLHV VQKEWMRLCT SVTESEVARA RNLLKTNMLL QLDGSTPICE 430 440 450 460 470 480 DIGRQMLCYN RRIPIPELEA RIDAVNAETI REVCTKYIYN RSPAIAAVGP IKQLPDFKQI RSNMCWLRD

References

[1]	"Identification of genes expressed in human CD34(+) hematopoietic stem/progenitor cells by expressed sequence tags and efficient full-length cDNA cloning." Mao M., Fu G., Wu J.-S., Zhang Q.-H., Zhou J., Kan L.-X., Huang Q.-H., He K.-L., Gu B.-W., Han Z.-G., Shen Y., Gu J., Yu Y.-P., Xu S.-H., Wang Y.-X., Chen S.-J., Chen Z. Proc. Natl. Acad. Sci. U.S.A. 95:8175-8180(1998) [PubMed: 9653160] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Tissue: Umbilical cord blood.
[2]	"The DNA sequence of human chromosome 7." Hillier L.W., Fulton R.S., Fulton L.A., Graves T.A., Pepin K.H., Wagner-McPherson C., Layman D., Maas J., Jaeger S., Walker R., Wylie K., Sekhon M., Becker M.C., O'Laughlin M.D., Schaller M.E., Fewell G.A., Delehaunty K.D., Miner T.L. , Nash W.E., Cordes M., Du H., Sun H., Edwards J., Bradshaw-Cordum H., Ali J., Andrews S., Isak A., Vanbrunt A., Nguyen C., Du F., Lamar B., Courtney L., Kalicki J., Ozersky P., Bielicki L., Scott K., Holmes A., Harkins R., Harris A., Strong C.M., Hou S., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Leonard S., Rohlfing T., Rock S.M., Tin-Wollam A.-M., Abbott A., Minx P., Maupin R., Strowmatt C., Latreille P., Miller N., Johnson D., Murray J., Woessner J.P., Wendl M.C., Yang S.-P., Schultz B.R., Wallis J.W., Spieth J., Bieri T.A., Nelson J.O., Berkowicz N., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Bedell J.A., Mardis E.R., Clifton S.W., Chissoe S.L., Marra M.A., Raymond C., Haugen E., Gillett W., Zhou Y., James R., Phelps K., Iadanoto S., Bubb K., Simms E., Levy R., Clendenning J., Kaul R., Kent W.J., Furey T.S., Baertsch R.A., Brent M.R., Keibler E., Flicek P., Bork P., Suyama M., Bailey J.A., Portnoy M.E., Torrents D., Chinwalla A.T., Gish W.R., Eddy S.R., McPherson J.D., Olson M.V., Eichler E.E., Green E.D., Waterston R.H., Wilson R.K. Nature 424:157-164(2003) [PubMed: 12853948] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[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: Skeletal muscle.
[4]	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	AF054182 mRNA. Translation: AAC39915.1. AC004668 Genomic DNA. No translation available. BC010398 mRNA. Translation: AAH10398.1.
IPI	IPI00927892.
RefSeq	NP_004270.2.
UniGene	Hs.184211
3D structure databases
HSSP	HSSP built from PDB template 1HR6 based on UniProtKB P10507.
ModBase	Search...
Protein-protein interaction databases
IntAct	O75439. 1 interaction.
Proteomic databases
PRIDE	O75439.
Genome annotation databases
Ensembl	ENSG00000105819. Homo sapiens. [Contig view]
GeneID	9512.
KEGG	hsa:9512.
NMPDR	fig|9606.3.peg.29229.
UCSC	uc003vbk.1. human.
Organism-specific databases
GeneCards	GC07P102725.
HGNC	HGNC:9119. PMPCB.
MIM	603131. gene.
PharmGKB	PA33445.
GenAtlas	Search...
Phylogenomic databases
HOVERGEN	O75439.
OMA	O75439. IPLMVAN.
Enzyme and pathway databases
BRENDA	3.4.24.64. 247.
Gene expression databases
ArrayExpress	O75439.
Bgee	O75439.
CleanEx	HS_PMPCB.
GermOnline	ENSG00000105819. 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	35642.
SOURCE	Search...

Entry information

Entry name

MPPB_HUMAN

Accession

Primary (citable) accession number: O75439
Secondary accession number(s): O60416, Q96FV4

Entry history

Integrated into UniProtKB/Swiss-Prot:	December 15, 1998
Last sequence update:	April 11, 2003
Last modified:	July 7, 2009
This is version 83 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)