TCDB is operated by the Saier Lab Bioinformatics Group
« See all members of the family


3.A.2.2.4
The ubiquitous 14 subunit vacuolar H+-ATPase, V1/V0, has been implicated in various human diseases including osteopetrosis, renal tubule acidosis, and cancer (Hinton et al., 2009).  The transmembrane enzyme, Ribonuclease kappa, RNASEK (137 aas; 2 TMSs; UniProt acc# Q6P5S7), closely associates with the V-ATPase and is required for its function; its loss prevents the early events of endocytosis and the replication of multiple pathogenic viruses (Perreira et al. 2015).

Accession Number:P15313
Protein Name:V-type proton ATPase subunit B, kidney isoform
Length:513
Molecular Weight:56833.00
Species: [9606]
Location1 / Topology2 / Orientation3: Endomembrane system1 / Peripheral membrane protein2
Substrate H+

Cross database links:

Genevestigator: P15313
eggNOG: prNOG12784
HEGENOM: HBG565875
RefSeq: NP_001683.2   
Entrez Gene ID: 525   
Pfam: PF00006    PF00306    PF02874   
Drugbank: Drugbank Link   
OMIM: 192132  gene
267300  phenotype
KEGG: hsa:525   

Gene Ontology

GO:0016324 C:apical plasma membrane
GO:0012505 C:endomembrane system
GO:0033180 C:proton-transporting V-type ATPase, V1 domain
GO:0016471 C:vacuolar proton-transporting V-type ATPase ...
GO:0046933 F:hydrogen ion transporting ATP synthase acti...
GO:0046961 F:proton-transporting ATPase activity, rotati...
GO:0015986 P:ATP synthesis coupled proton transport
GO:0007588 P:excretion
GO:0001503 P:ossification
GO:0006885 P:regulation of pH
GO:0007605 P:sensory perception of sound

References (7)

[1] “Human endomembrane H+ pump strongly resembles the ATP-synthetase of Archaebacteria.”  Suedhof T.C.et.al.   2527371
[2] “Complete sequencing and characterization of 21,243 full-length human cDNAs.”  Ota T.et.al.   14702039
[3] “The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).”  The MGC Project Teamet.al.   15489334
[4] “The COOH termini of NBC3 and the 56-kDa H+-ATPase subunit are PDZ motifs involved in their interaction.”  Pushkin A.et.al.   12444018
[5] “Mutations in the gene encoding B1 subunit of H+-ATPase cause renal tubular acidosis with sensorineural deafness.”  Karet F.E.et.al.   9916796
[6] “Novel ATP6V1B1 and ATP6V0A4 mutations in autosomal recessive distal renal tubular acidosis with new evidence for hearing loss.”  Stover E.H.et.al.   12414817
[7] “Confirmation of the ATP6B1 gene as responsible for distal renal tubular acidosis.”  Ruf R.et.al.   12579397

External Searches:

  • Search: DB with
  • BLAST ExPASy (Swiss Institute of Bioinformatics (SIB) BLAST)
  • CDD Search (Conserved Domain Database)
  • Search COGs (Clusters of Orthologous Groups of proteins)
  • 2° Structure (Network Protein Sequence Analysis)

Analyze:

Predict TMSs (Predict number of transmembrane segments)
Window Size: Angle:  
Window Size: Angle:  
FASTA formatted sequence
1:	MAMEIDSRPG GLPGSSCNLG AAREHMQAVT RNYITHPRVT YRTVCSVNGP LVVLDRVKFA 
61:	QYAEIVHFTL PDGTQRSGQV LEVAGTKAIV QVFEGTSGID ARKTTCEFTG DILRTPVSED 
121:	MLGRVFNGSG KPIDKGPVVM AEDFLDINGQ PINPHSRIYP EEMIQTGISP IDVMNSIARG 
181:	QKIPIFSAAG LPHNEIAAQI CRQAGLVKKS KAVLDYHDDN FAIVFAAMGV NMETARFFKS 
241:	DFEQNGTMGN VCLFLNLAND PTIERIITPR LALTTAEFLA YQCEKHVLVI LTDMSSYAEA 
301:	LREVSAAREE VPGRRGFPGY MYTDLATIYE RAGRVEGRGG SITQIPILTM PNDDITHPIP 
361:	DLTGFITEGQ IYVDRQLHNR QIYPPINVLP SLSRLMKSAI GEGMTRKDHG DVSNQLYACY 
421:	AIGKDVQAMK AVVGEEALTS EDLLYLEFLQ KFEKNFINQG PYENRSVFES LDLGWKLLRI 
481:	FPKEMLKRIP QAVIDEFYSR EGALQDLAPD TAL