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Protein

Protein Mpv17

Gene

Mpv17

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at transcript leveli

Functioni

Involved in mitochondria homeostasis. May be involved in the metabolism of reactive oxygen species and control of oxidative phosphorylation and mitochondrial DNA (mtDNA) maintenance (Probable).1 Publication

GO - Biological processi

  1. cellular response to reactive oxygen species Source: UniProtKB
  2. glomerular basement membrane development Source: UniProtKB
  3. homeostatic process Source: MGI
  4. inner ear development Source: UniProtKB
  5. mitochondrial genome maintenance Source: MGI
  6. reactive oxygen species metabolic process Source: MGI
  7. regulation of reactive oxygen species metabolic process Source: UniProtKB
Complete GO annotation...

Names & Taxonomyi

Protein namesi
Recommended name:
Protein Mpv17
Short name:
Mpv-17
Gene namesi
Name:Mpv17
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589: Chromosome 5

Organism-specific databases

MGIiMGI:97138. Mpv17.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transmembranei18 – 3821HelicalSequence AnalysisAdd
BLAST
Transmembranei53 – 7321HelicalSequence AnalysisAdd
BLAST
Transmembranei94 – 11421HelicalSequence AnalysisAdd
BLAST
Transmembranei131 – 15121HelicalSequence AnalysisAdd
BLAST

GO - Cellular componenti

  1. integral component of membrane Source: UniProtKB-KW
  2. mitochondrial inner membrane Source: MGI
  3. mitochondrion Source: MGI
  4. peroxisome Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Disruption phenotypei

Mice lack expression of this protein resulting in the development of adult onset nephrotic syndrome and chronic renal failure. They also develop severe morphological degeneration of the inner ear. In the inner ear, mice lacking Mpv17 display degenerative changes of the cochlear structures already at the age of 2 months. The degenerative changes are patchy arranged throughout the entire length of the cochlea and involved the organ of Corti as well as the stria vascularis epithelia with alterations of the basement membrane of the capillaries.2 Publications

Keywords - Diseasei

Deafness

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 176176Protein Mpv17PRO_0000218928Add
BLAST

Proteomic databases

MaxQBiP19258.
PaxDbiP19258.
PRIDEiP19258.

Expressioni

Tissue specificityi

High levels in heart, kidney, and brain, intermediate levels in testis, and low levels in liver and spleen.

Gene expression databases

BgeeiP19258.
CleanExiMM_MPV17.
ExpressionAtlasiP19258. baseline and differential.
GenevestigatoriP19258.

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiNOG44594.
GeneTreeiENSGT00530000063005.
HOGENOMiHOG000039675.
HOVERGENiHBG105417.
InParanoidiP19258.
KOiK13348.
OrthoDBiEOG7TQV25.
TreeFamiTF324070.

Family and domain databases

InterProiIPR007248. Mpv17_PMP22.
[Graphical view]
PANTHERiPTHR11266. PTHR11266. 1 hit.
PfamiPF04117. Mpv17_PMP22. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

P19258-1 [UniParc]FASTAAdd to Basket

« Hide

        10         20         30         40         50
MALWRAYQRA LAAHPWKVQV LTAGSLMGVG DMISQQLVER RGLQQHQAGR
60 70 80 90 100
TLTMVSLGCG FVGPVVGGWY KVLDHLIPGT TKVHALKKML LDQGGFAPCF
110 120 130 140 150
LGCFLPLVGI LNGMSAQDNW AKLKRDYPDA LITNYYLWPA VQLANFYLVP
160 170
LHYRLAVVQC VAIVWNSYLS WKAHQF
Length:176
Mass (Da):19,686
Last modified:November 1, 1990 - v1
Checksum:i7060A7B122F77DB1
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M36411 mRNA. Translation: AAA39736.1.
BC013452 mRNA. Translation: AAH13452.1.
CCDSiCCDS19175.1.
PIRiS29031.
RefSeqiNP_001281253.1. NM_001294324.1.
NP_032648.1. NM_008622.6.
XP_006503833.1. XM_006503770.1.
UniGeneiMm.435472.

Genome annotation databases

EnsembliENSMUST00000114631; ENSMUSP00000110278; ENSMUSG00000090262.
ENSMUST00000114632; ENSMUSP00000110279; ENSMUSG00000090262.
ENSMUST00000154241; ENSMUSP00000115292; ENSMUSG00000090262.
GeneIDi17527.
KEGGimmu:17527.
UCSCiuc008wxf.2. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M36411 mRNA. Translation: AAA39736.1.
BC013452 mRNA. Translation: AAH13452.1.
CCDSiCCDS19175.1.
PIRiS29031.
RefSeqiNP_001281253.1. NM_001294324.1.
NP_032648.1. NM_008622.6.
XP_006503833.1. XM_006503770.1.
UniGeneiMm.435472.

3D structure databases

ModBaseiSearch...
MobiDBiSearch...

Proteomic databases

MaxQBiP19258.
PaxDbiP19258.
PRIDEiP19258.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000114631; ENSMUSP00000110278; ENSMUSG00000090262.
ENSMUST00000114632; ENSMUSP00000110279; ENSMUSG00000090262.
ENSMUST00000154241; ENSMUSP00000115292; ENSMUSG00000090262.
GeneIDi17527.
KEGGimmu:17527.
UCSCiuc008wxf.2. mouse.

Organism-specific databases

CTDi4358.
MGIiMGI:97138. Mpv17.

Phylogenomic databases

eggNOGiNOG44594.
GeneTreeiENSGT00530000063005.
HOGENOMiHOG000039675.
HOVERGENiHBG105417.
InParanoidiP19258.
KOiK13348.
OrthoDBiEOG7TQV25.
TreeFamiTF324070.

Miscellaneous databases

NextBioi292140.
PROiP19258.
SOURCEiSearch...

Gene expression databases

BgeeiP19258.
CleanExiMM_MPV17.
ExpressionAtlasiP19258. baseline and differential.
GenevestigatoriP19258.

Family and domain databases

InterProiIPR007248. Mpv17_PMP22.
[Graphical view]
PANTHERiPTHR11266. PTHR11266. 1 hit.
PfamiPF04117. Mpv17_PMP22. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Transgenic mouse model of kidney disease: insertional inactivation of ubiquitously expressed gene leads to nephrotic syndrome."
    Weiher H., Noda T., Gray D.A., Sharpe A.H., Jaenisch R.
    Cell 62:425-434(1990) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
  2. "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] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Strain: FVB/N.
    Tissue: Kidney.
  3. "The glomerulosclerosis gene Mpv17 encodes a peroxisomal protein producing reactive oxygen species."
    Zwacka R.M., Reuter A., Pfaff E., Moll J., Gorgas K., Karasawa M., Weiher H.
    EMBO J. 13:5129-5134(1994) [PubMed] [Europe PMC] [Abstract]
    Cited for: PRELIMINARY SUBCELLULAR LOCATION, FUNCTION.
  4. Cited for: DISRUPTION PHENOTYPE.
  5. "Ultrastructural and physiological defects in the cochlea of the Mpv17 mouse strain. A comparison between young and old adult animals."
    Meyer zum Gottesberge A.M., Felix H., Reuter A., Weiher H.
    Hear. Res. 156:69-80(2001) [PubMed] [Europe PMC] [Abstract]
    Cited for: DISRUPTION PHENOTYPE.

Entry informationi

Entry nameiMPV17_MOUSE
AccessioniPrimary (citable) accession number: P19258
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1990
Last sequence update: November 1, 1990
Last modified: February 4, 2015
This is version 113 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Caution

Was initially thought to be a peroxisomal protein (PubMed:7957077). However, it was later shown in human that it is a mitochondrial protein (PubMed:16582907 and PubMed:16582910).1 Publication

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.