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Surfeit 1 (SURF1)

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Surfeit Locus Protein 1

Surfeit 1 (SURF1)
CCC C880
HGNC 11474
MGI 98443
PubMed 10558868
RGD 620527
UniProt Q15526
Wiki SURF1
Surfeit locus protein 1 is a protein localized to the inner mitochondrial membrane and thought to be involved in the biogenesis of the cytochrome c oxidase complex. The protein is a member of the SURF1 family, which includes the related yeast protein SHY1 and rickettsial protein RP733.
The gene is located in the surfeit gene cluster, a group of very tightly linked genes that do not share sequence similarity, where it shares a bidirectional promoter with SURF2 on the opposite strand. Defects in this gene are a cause of Leigh syndrome, a severe neurological disorder that is commonly associated with systemic cytochrome c oxidase deficiency.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Surfeit 1 (SURF1) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Surfeit 1 (SURF1) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Surfeit 1 (SURF1) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Surfeit 1 (SURF1) CLIA Kit Customized Service Offer
n/a ELISA Kit for Surfeit 1 (SURF1) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Surfeit 1 (SURF1) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Surfeit 1 (SURF1) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Surfeit 1 (SURF1) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Surfeit 1 (SURF1) CLIA Kit Customized Service Offer
n/a ELISA Kit for Surfeit 1 (SURF1) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPC880Ra01 Recombinant Surfeit 1 (SURF1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAC880Ra01 Polyclonal Antibody to Surfeit 1 (SURF1) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Surfeit 1 (SURF1) CLIA Kit Customized Service Offer
n/a ELISA Kit for Surfeit 1 (SURF1) ELISA Kit Customized Service Offer
  1. "The Surf-1 and Surf-2 genes and their essential bidirectional promoter elements are conserved between mouse and human."DNA Cell Biol. 13:1117-1126(1994) [PubMed] [Europe PMC] [Abstract]
  2. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  3. "DNA sequence and analysis of human chromosome 9." Nature 429:369-374(2004) [PubMed] [Europe PMC] [Abstract]
  4. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  5. "SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome."Nat. Genet. 20:337-343(1998) [PubMed] [Europe PMC] [Abstract]
  6. "Mutations in the SURF1 gene associated with Leigh syndrome and cytochrome C oxidase deficiency."Hum. Mutat. 17:374-381(2001) [PubMed] [Europe PMC] [Abstract]
  7. "Missense mutations in SURF1 associated with deficient cytochrome c oxidase assembly in Leigh syndrome patients."Hum. Genet. 106:194-205(2000) [PubMed] [Europe PMC] [Abstract]
  8. "Two novel mutations of SURF1 in Leigh syndrome with cytochrome c oxidase deficiency."Hum. Genet. 105:560-563(1999) [PubMed] [Europe PMC] [Abstract]
  9. "SURF1 gene mutations in Polish patients with COX-deficient Leigh syndrome."J. Appl. Genet. 42:103-108(2001) [PubMed] [Europe PMC] [Abstract]
  10. "The consensus coding sequences of human breast and colorectal cancers." Science 314:268-274(2006) [PubMed] [Europe PMC] [Abstract]
  11. "Deep sequencing reveals 50 novel genes for recessive cognitive disorders." Nature 478:57-63(2011) [PubMed] [Europe PMC] [Abstract]
  12. "Two Japanese patients with Leigh syndrome caused by novel SURF1 mutations."Brain Dev. 34:861-865(2012) [PubMed] [Europe PMC] [Abstract]
  13. "SURF1-associated leigh syndrome: A case series and novel mutations."Hum. Mutat. 33:1192-1200(2012) [PubMed] [Europe PMC] [Abstract]