anti-BCS1-Like (S. Cerevisiae) (BCS1L) Anticorps

BCS1L encodes a homolog of the S. De plus, nous expédions BCS1-Like (S. Cerevisiae) Protéines (8) et BCS1-Like (S. Cerevisiae) Kits (6) et beaucoup plus de produits pour cette protéine.

afficher tous les anticorps Gène GeneID UniProt
BCS1L 617 Q9Y276
BCS1L 66821 Q9CZP5
BCS1L 301514  
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Top anti-BCS1-Like (S. Cerevisiae) Anticorps sur anticorps-enligne.fr

Showing 10 out of 25 products:

Catalogue No. Reactivité Hôte Conjugué Application Images Quantité Livraison Prix Détails
Humain Lapin Inconjugué IHC, WB Western blot analysis of extracts from HepG2, using BCS1L antibody. Lane 1 was treated with the blocking peptide. ABIN6273033 at 1/100 staining Human breast cancer tissue by IHC-P. The sample was formaldehyde fixed and a heat mediated antigen retrieval step in citrate buffer was performed. The sample was then blocked and incubated with the antibody for 1.5 hours at 22°C. An HRP conjugated goat anti-rabbit antibody was used as the secondary. 100 μL 11 to 12 Days
$390.77
Détails
Humain Souris Inconjugué ELISA, WB Western Blot analysis of BCS1L expression in transfected 293T cell line by BCS1L monoclonal antibody (M01), clone 5F3.Lane 1: BCS1L transfected lysate(47.534 KDa).Lane 2: Non-transfected lysate. Immunoprecipitation of BCS1L transfected lysate using anti-BCS1L monoclonal antibody and Protein A Magnetic Bead , and immunoblotted with BCS1L MaxPab rabbit polyclonal antibody. 100 μg 11 to 12 Days
$364.00
Détails
Humain Souris Inconjugué WB Western Blot analysis of BCS1L expression in transfected 293T cell line by BCS1L MaxPab polyclonal antibody.Lane 1: BCS1L transfected lysate(46.09 KDa).Lane 2: Non-transfected lysate. 50 μL 11 to 12 Days
$364.00
Détails
Humain Lapin Inconjugué IHC, WB Western blot analysis of extracts of various cell lines, using BCS1L antibody. Immunohistochemistry of paraffin-embedded human stomach using BCS1L antibody. 100 μL 13 to 14 Days
$393.25
Détails
Humain Lapin Inconjugué WB Western Blot analysis of BCS1L expression in transfected 293T cell line by BCS1L MaxPab polyclonal antibody.Lane 1: BCS1L transfected lysate(47.50 KDa).Lane 2: Non-transfected lysate. BCS1L MaxPab rabbit polyclonal antibody. Western Blot analysis of BCS1L expression in mouse spleen. 100 μg 11 to 12 Days
$350.67
Détails
Humain Lapin Inconjugué IHC, WB 100 μL 11 to 18 Days
$383.76
Détails
Humain Lapin Inconjugué WB   100 μg 11 to 14 Days
$903.83
Détails
Humain Lapin Inconjugué IF, IHC, WB Western blot analysis of extracts of various cell lines, using BCS1L antibody. 100 μL 6 to 11 Days
$390.55
Détails
Humain Souris Inconjugué ELISA, IF, IHC, WB   100 μg 13 to 16 Days
$407.69
Détails
Humain Lapin Inconjugué ELISA, IHC, WB   100 μg 13 to 16 Days
$407.69
Détails

anti-BCS1-Like (S. Cerevisiae) Anticorps mieux référencés

  1. Human Monoclonal BCS1L Primary Antibody pour ELISA, WB - ABIN560048 : Gil-Borlado, González-Hoyuela, Blázquez, García-Silva, Gabaldón, Manzanares, Vara, Martín, Seneca, Arenas, Ugalde: Pathogenic mutations in the 5' untranslated region of BCS1L mRNA in mitochondrial complex III deficiency. dans Mitochondrion 2009 (PubMed)
    Show all 2 Pubmed References

Plus d’anticorps contre BCS1-Like (S. Cerevisiae) partenaires d’interaction

Human BCS1-Like (S. Cerevisiae) (BCS1L) interaction partners

  1. The BCSIL gene mutation is responsible for GRACILE syndrome, Bjornstad syndrome and complex III deficiency. Bjomstad syndrome is characterized by sensorineural hearing loss and abnormal flat twisted hair shafts. The case is GRACILE syndrome with Bjomstad phenotype in neonatal period due to BCSL1 gene mutation.

  2. We report the first Italian patients with Bjornstad syndrome, two siblings with pili torti and sensorineural hearing loss, in whom we detected two novel compound heterozygous mutations in BCS1L

  3. * Description of a novel homozygous mutation in BCS1L with transient neonatal acidosis and persistent de Toni-Debre-Fanconi-type tubulopathy. * The long survival of patients with phenotypic presentation of severe complex III deficiency is uncommon.

  4. Extensive statistical and cluster analyses revealed a protein profile characteristic for the BCS1L mutant fibroblasts that included alterations in energy metabolism, cell signaling and gene expression regulation, cytoskeleton formation and maintenance.

  5. Exome sequencing revealed novel BCS1L mutations in two siblings with Bjornstad syndrome characterized by hearing loss and hypotrichosis.

  6. A novel behavioral and psychiatric phenotype associated with a p.Gly129Arg BCS1L mutation.

  7. This region encompasses the BCS1L gene.

  8. These results provide new insights into the role of pathogenic BCS1L mutations in mitochondrial function and dynamics.

  9. GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L

  10. a function of BCS1L is to promote the maturation of complex III and the incorporation of the Rieske iron-sulfur protein into the nascent complex. Defective BCS1L leads to the formation of a catalytically inactive, structurally unstable complex III.

  11. assessed whether 232A-->G or other BCS1L mutations were present in infants (n = 21) of Finnish origin with severe, lethal disease compatible with mitochondrial disorder

  12. BCS1L stimulates the assembly of the LETM1 complex. BCS1L knockdown caused disassembly of the respiratory chains as well as LETM1 downregulation and induced distinct changes in mitochondrial morphology.

  13. The severity of the complex III enzyme defect correlated with decreased amounts of BCS1L and respiratory chain complex III. This supports a pathogenic role for the novel BCS1L mutation in a patient with a singular clinical phenotype.

  14. The g.1181A>G mutation generated an alternative splicing site in the BCS1L transcript, causing a 19-nucleotides deletion in its 5'UTR region and Complex III deficiency.

  15. mitochondrial complex III deficiency caused by mutations in the BCS1L gene

Mouse (Murine) BCS1-Like (S. Cerevisiae) (BCS1L) interaction partners

  1. COX7A2L/SCAFI and Pre-Complex III Modify Respiratory Chain Supercomplex Formation in Different Mouse Strains with a Bcs1l Mutation.

  2. Studies in the yeast BCS1 ortholog indicate that the Bcs1 protein is anchored in the mitochondrial inner membrane, despite the absence of an N-terminal targeting sequence. Targeting occurs via charged amino acids near the transmembrane domain that act as an internal targeting signal.

  3. Metabolite profiles reveal energy failure and impaired beta-oxidation in liver of mice with complex III deficiency due to a BCS1L mutation.

  4. The GRACILE mutation introduced into Bcs1l causes postnatal complex III deficiency and is a viable mouse model for mitochondrial hepatopathy.

  5. The predominant expression of BCS1L in the floor plate of the neural tube region, together with its presence in peripheral ganglia from E13 onwards, indicates a role for BCS1L in the development of neural structures.

BCS1-Like (S. Cerevisiae) (BCS1L) profil antigène

Profil protéine

This gene encodes a homolog of the S. cerevisiae bcs1 protein which is involved in the assembly of complex III of the mitochondrial respiratory chain. The encoded protein does not contain a mitochondrial targeting sequence but experimental studies confirm that it is imported into mitochondria. Mutations in this gene are associated with mitochondrial complex III deficiency and the GRACILE syndrome. Five alternatively spliced transcripts encoding the same protein have been described.

Gene names and symbols associated with BCS1L

  • BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone (BCS1L) anticorps
  • mitochondrial chaperone BCS1 (AFUA_3G13000) anticorps
  • mitochondrial chaperone bcs1 (CC1G_00820) anticorps
  • mitochondrial chaperone BCS1 (CC1G_01181) anticorps
  • mitochondrial chaperone BCS1 (CC1G_01608) anticorps
  • mitochondrial chaperone BCS1 (CC1G_11035) anticorps
  • mitochondrial chaperone BCS1 (CC1G_11036) anticorps
  • mitochondrial chaperone BCS1 (Bm1_30135) anticorps
  • mitochondrial ATPase (bcs1lA) anticorps
  • mitochondrial ATPase (bcs1lB) anticorps
  • BCS1-like (yeast) (Bcs1l) anticorps
  • BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone L homeolog (bcs1l.L) anticorps
  • BC1 (ubiquinol-cytochrome c reductase) synthesis-like (bcs1l) anticorps
  • BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone (Bcs1l) anticorps
  • 9130022O19Rik anticorps
  • BCS anticorps
  • BCS1 anticorps
  • BJS anticorps
  • DDBDRAFT_0184131 anticorps
  • DDBDRAFT_0188274 anticorps
  • DDBDRAFT_0266725 anticorps
  • DDBDRAFT_0266726 anticorps
  • DDB_0184131 anticorps
  • DDB_0188274 anticorps
  • DDB_0266725 anticorps
  • DDB_0266726 anticorps
  • FLNMS anticorps
  • GRACILE anticorps
  • h-BCS anticorps
  • Hs.6719 anticorps
  • MC3DN1 anticorps
  • PTD anticorps
  • zgc:56205 anticorps

Protein level used designations for BCS1L

BCS1-like protein , h-BCS1 , mitochondrial chaperone BCS1 , mitochondrial complex III assembly , mitochondrial chaperone bcs1 , BC1 (ubiquinol-cytochrome c reductase) synthesis-like

GENE ID SPECIES
617 Homo sapiens
3512380 Aspergillus fumigatus Af293
6007738 Coprinopsis cinerea okayama7130
6009613 Coprinopsis cinerea okayama7130
6010435 Coprinopsis cinerea okayama7130
6010570 Coprinopsis cinerea okayama7130
6010571 Coprinopsis cinerea okayama7130
6100920 Brugia malayi
8626976 Dictyostelium discoideum AX4
8628408 Dictyostelium discoideum AX4
66821 Mus musculus
539713 Bos taurus
380366 Xenopus laevis
394157 Danio rerio
301514 Rattus norvegicus
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