SURF1 Proteine (SURF1)

Surfeit 1 Proteine (SURF1)
Auf finden Sie aktuell 10 Surfeit 1 (SURF1) Proteine von 5 unterschiedlichen Herstellern. Zusätzlich bieten wir Ihnen SURF1 Antikörper (31) und SURF1 Kits (1) und viele weitere Produktgruppen zu diesem Protein an. Insgesamt sind aktuell 48 SURF1 Produkte verfügbar.
0610010F23Rik, Ab1-205, BcDNA:GH23574, CG9943, Dmel\\CG9943, GB14201, im:6898613, Surf-1, SURF1, SURFEIT 1, zgc:158646
alle Proteine anzeigen Gen GeneID UniProt
SURF1 6834 Q15526
SURF1 64463 Q9QXU2
SURF1 20930 P09925

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Am meisten referenzierte SURF1 Proteine

  1. Human SURF1 Protein expressed in Escherichia coli (E. coli) - ABIN666926 : Péquignot, Dey, Zeviani, Tiranti, Godinot, Poyau, Sue, Di Mauro, Abitbol, Marsac: Mutations in the SURF1 gene associated with Leigh syndrome and cytochrome C oxidase deficiency. in Human mutation 2001 (PubMed)
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Human Surfeit 1 (SURF1) Interaktionspartner

  1. Mutations in the SURF1 gene are a cause of Charcot-Marie-Tooth disease.

  2. This study suggested that hypertrophic olivary degeneration on magnetic resonance imaging in mitochondrial syndromes associated with POLG (zeige POLG Proteine) and SURF1 mutations.

  3. sequenced the SURF1 gene and identified two heterozygous mutations; c.49+1 G>T and c.752_753del in Case 1, and homozygous c.743 C>A in Case 2

  4. Study identified 21 patients with clinical features of Leigh syndrome who are either homozygous or compound heterozygous for SURF1 mutations.

  5. Analysis of fibroblast cell lines from 9 patients with SURF1 mutations revealed a 70% decrease of the COX (zeige COX8A Proteine) complex content to be associated with 32-54% upregulation of respiratory chain complexes I, III and V and accumulation of Cox5a (zeige COX5A Proteine) subunit.

  6. Analysis of mutations in the SURF1 homolog Shy1 revealed Coa4, a new member of the cytochrome oxidase assembly factor family.

  7. Three novel mutations of the SURF-1 gene were identified in Japanese patients with cytochrome c (zeige CYCS Proteine) oxidase deficiency; loss of function of the SURF-1 protein; cytochrome c (zeige CYCS Proteine) oxidase activity was decreased to less than 20% of the control mean.

  8. new missense mutation of 574C>T in the SURF1 gene in Leigh's syndrome

  9. Two novel pathogenic SURF1 mutations have been identified in a patient with Leigh syndrome.

  10. Mutations in the nuclear SURF1 gene are specifically associated with cytochrome c (zeige CYCS Proteine) oxidase (COX (zeige COX8A Proteine))-deficient Leigh syndrome. MR imaging abnormalities in three children with this condition involved the brain.

Mouse (Murine) Surfeit 1 (SURF1) Interaktionspartner

  1. These findings suggest that Surf1 deficiency-induced metabolic alterations may have positive effects on brain function.

  2. The expression of peroxisome proliferator-activated receptor gamma-coactivator 1-alpha (PGC-1alpha) mRNA and protein was up-regulated in white adipose tissue from Surf1-/- mice, and the expression of PGC-1alpha target genes

  3. Compared to wild type (WT) preparations Surf1 knockout preparations had a higher baseline respiratory frequency and abnormal responses to hypoxia and hypercapnia that involved both respiratory frequency and motor nerve discharge pattern.

  4. murine Surf1 protein (Surf1p) has a function specifically related to cytochrome c (zeige CYCS Proteine) oxidase

  5. Prolonged lifespan and complete protection from Ca(2 (zeige CA2 Proteine)+)-dependent neurotoxicity induced by kainic acid in Surf-1 knockout mice.

SURF1 Protein Überblick

Protein Überblick

This gene encodes 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.

Alternative names and synonyms associated with SURF1

  • Surfeit 1 (Surf1)
  • surfeit 1 (Surf1)
  • surfeit 1 (SURF1)
  • surfeit 1 (surf1)
  • surfeit gene 1 (Surf1)
  • Surfeit locus 1 cytochrome c oxidase biogenesis protein (SURF1)
  • 0610010F23Rik Protein
  • Ab1-205 Protein
  • BcDNA:GH23574 Protein
  • CG9943 Protein
  • Dmel\\CG9943 Protein
  • GB14201 Protein
  • im:6898613 Protein
  • Surf-1 Protein
  • SURF1 Protein
  • SURFEIT 1 Protein
  • zgc:158646 Protein

Bezeichner auf Proteinebene für Surfeit 1 Proteine (SURF1)

CG9943-PA , Surf1-PA , surfeit 1 , surfeit locus protein 1 , surfeit protein

44498 Drosophila melanogaster
413781 Apis mellifera
480681 Canis lupus familiaris
557970 Danio rerio
693110 Bombyx mori
6834 Homo sapiens
64463 Rattus norvegicus
417157 Gallus gallus
20930 Mus musculus
821310 Arabidopsis thaliana
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