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P19258 (MPV17_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 109. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (2) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Protein Mpv17

Short name=Mpv-17
Gene names
Name:Mpv17
OrganismMus musculus (Mouse) [Reference proteome]
Taxonomic identifier10090 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus

Protein attributes

Sequence length176 AA.
Sequence statusComplete.
Protein existenceEvidence at transcript level

General annotation (Comments)

Function

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. Ref.3

Subcellular location

Mitochondrion inner membrane; Multi-pass membrane protein By similarity Ref.3.

Tissue specificity

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

Disruption phenotype

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. Ref.4 Ref.5

Sequence similarities

Belongs to the peroxisomal membrane protein PXMP2/4 family.

Caution

Was initially thought to be a peroxisomal protein (Ref.3). However, it was later shown in human that it is a mitochondrial protein (PubMed:16582907 and PubMed:16582910).

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 176176Protein Mpv17
PRO_0000218928

Regions

Transmembrane18 – 3821Helical; Potential
Transmembrane53 – 7321Helical; Potential
Transmembrane94 – 11421Helical; Potential
Transmembrane131 – 15121Helical; Potential

Sequences

Sequence LengthMass (Da)Tools
P19258 [UniParc].

Last modified November 1, 1990. Version 1.
Checksum: 7060A7B122F77DB1

FASTA17619,686
        10         20         30         40         50         60 
MALWRAYQRA LAAHPWKVQV LTAGSLMGVG DMISQQLVER RGLQQHQAGR TLTMVSLGCG 

        70         80         90        100        110        120 
FVGPVVGGWY KVLDHLIPGT TKVHALKKML LDQGGFAPCF LGCFLPLVGI LNGMSAQDNW 

       130        140        150        160        170 
AKLKRDYPDA LITNYYLWPA VQLANFYLVP LHYRLAVVQC VAIVWNSYLS WKAHQF 

« Hide

References

« Hide 'large scale' references
[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]"Loss of auditory function in transgenic Mpv17-deficient mice."
Muller M., Smolders J.W.T., Meyer zum Gottesberge A.M., Reuter A., Zwacka R.M., Weiher H., Klinke R.
Hear. Res. 114:259-263(1997) [PubMed] [Europe PMC] [Abstract]
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.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
M36411 mRNA. Translation: AAA39736.1.
BC013452 mRNA. Translation: AAH13452.1.
CCDSCCDS19175.1.
PIRS29031.
RefSeqNP_032648.1. NM_008622.5.
XP_006503833.1. XM_006503770.1.
UniGeneMm.435472.

3D structure databases

ModBaseSearch...
MobiDBSearch...

Proteomic databases

PaxDbP19258.
PRIDEP19258.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENSMUST00000114631; ENSMUSP00000110278; ENSMUSG00000090262.
ENSMUST00000114632; ENSMUSP00000110279; ENSMUSG00000090262.
ENSMUST00000154241; ENSMUSP00000115292; ENSMUSG00000090262.
GeneID17527.
KEGGmmu:17527.
UCSCuc008wxf.2. mouse.

Organism-specific databases

CTD4358.
MGIMGI:97138. Mpv17.

Phylogenomic databases

eggNOGNOG44594.
GeneTreeENSGT00530000063005.
HOGENOMHOG000039675.
HOVERGENHBG105417.
KOK13348.
OrthoDBEOG7TQV25.
TreeFamTF324070.

Gene expression databases

BgeeP19258.
CleanExMM_MPV17.
GenevestigatorP19258.

Family and domain databases

InterProIPR007248. Mpv17_PMP22.
[Graphical view]
PANTHERPTHR11266. PTHR11266. 1 hit.
PfamPF04117. Mpv17_PMP22. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

NextBio292140.
PROP19258.
SOURCESearch...

Entry information

Entry nameMPV17_MOUSE
AccessionPrimary (citable) accession number: P19258
Entry history
Integrated into UniProtKB/Swiss-Prot: November 1, 1990
Last sequence update: November 1, 1990
Last modified: July 9, 2014
This is version 109 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families

MGD cross-references

Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot