Gene Name
Protein ID
Chromosome ID
HPP Status
Protein Name

Vitelliform macular dystrophy 2-like protein 3



bestrophin 3

vitelliform macular dystrophy 2-like 3

vitelliform macular dystrophy 2-like protein 3

[Reference: ]
Chromosomal Position
12q15 | Start:69643360 End:69643360

Entrez Gene Summary for BEST3
BEST3 belongs to the bestrophin family of anion channels, which includes BEST1 (MIM 607854), the gene mutant in vitelliform macular dystrophy (VMD; MIM 153700), and 2 other BEST1-like genes, BEST2 (MIM 607335) and BEST4 (MIM 607336). Bestrophins are transmembrane (TM) proteins that share a homology region containing a high content of aromatic residues, including an invariant arg-phe-pro (RFP) motif. The bestrophin genes share a conserved gene structure, with almost identical sizes of the 8 RFP-TM domain-encoding exons and highly conserved exon-intron boundaries. Each of the 4 bestrophin genes has a unique 3-prime end of variable length (Stohr et al., 2002 (PubMed 12032738); Tsunenari et al., 2003 (PubMed 12907679)).(supplied by OMIM, Mar 2008)

UniProtKB Summary for BEST3
Function: Forms calcium-sensitive (By similarity) chloride channels. Permeable to bicarbonate (By similarity)

[Reference: ]
External IDs
Hgnc ID: 17105 EntrezGene ID: 144453 Ensembl ID: ENSG00000127325
[Reference: ]
Reference Source

Gene Reference Into Function (GeneRIF)

PubMed IDGeneRIF TextLast Update
12032738identified three novel VMD2-related human genes demonstrating a high degree of conservation in their respective RFP-TM domains [VMD2L1, VMD2L2, VMD2L3]2010-01-21
17442670These results suggest that an auto-inhibitory mechanism in C termini of bestrophin 3 may be universal among bestrophins investigated in the study.2010-01-21
19237432Results provide evidence that the bestrophins are expressed in pancreatic duct cells and, more specifically, that hBest1 plays a role in the calcium activated chloride channels found in these cells.2010-01-21
25329324results demonstrated that Best-3 is an endogenous inhibitor of NF-kappaB signaling pathway in endothelial cells, suggesting that forced Best-3 expression may be a novel approach for the treatment of vascular inflammatory diseases.2015-07-04


Relevant citations within the PubMed literature


Putative/known Functions


Homologues, Orthologues, Paralogues and Family

Sequence Similarity and Functional Annotation

Sequence Similarity

Db NameQuery UniSubject UniSequence LengthAlignment LengthIdentityCoverageMismatchesGap OpeningsQuery StartQuery EndSubject StartSubject EndEvalueBit Score
Reviewed non-human mammalian with experimental evidenceBEST3_HUMANBEST1_PIG66821862.8432.6381015637332201E-82303
Islam MT, Garg G, Hancock WS, Risk BA, Baker MS, Ranganathan S (2014) Protannotator: A Semiautomated Pipeline for Chromosome-Wise Functional Annotation of the "Missing" Human Proteome. J Proteome Res. 13, 76-83.

Post Translational Modifications

Protein Protein Interactions

Best Available Mass Spectra without FDR


Evidence File
Fenyö, David; Beavis, Ronald C. (2015). "The GPMDB REST interface". Bioinformatics 31 (12): 2056–2058. doi:10.1093/bioinformatics/btv107. ISSN 1367-4803. ">Global Proteome Machine Database - THE GPM


Peptide SequenceScoresPride IDSpectrum IDAnnotation
No results found.
Evidence File
Vizcaino JA, et al. 2016 update of the PRIDE database and related tools. Nucleic Acids Res. 2016 Jan 1;44(D1):D447-D456. ">PRIDE Archive

Proteomics DB

Evidence File
Wilhelm, M et al. (2014) Mass-spectrometry-based draft of the human proteome. Nature. 509:582-7. ">Proteomics DB