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BCS1-Like (S. Cerevisiae) Proteins (BCS1L)

BCS1L encodes a homolog of the S. Additionally we are shipping BCS1-Like (S. Cerevisiae) Antibodies (20) and BCS1-Like (S. Cerevisiae) Kits (2) and many more products for this protein.

list all proteins Gene Name GeneID UniProt
BCS1L 617 Q9Y276
BCS1L 66821 Q9CZP5
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Top BCS1-Like (S. Cerevisiae) Proteins at antibodies-online.com

Showing 6 out of 8 products:

Catalog No. Origin Source Conjugate Images Quantity Supplier Delivery Price Details
HOST_Escherichia coli (E. coli) Human His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
Insect Cells Human rho-1D4 tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 0.5 mg Log in to see 49 to 54 Days
$6,041.49
Details
Insect Cells Mouse rho-1D4 tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 0.25 mg Log in to see 49 to 54 Days
$4,244.78
Details
HOST_Escherichia coli (E. coli) Mouse His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg Log in to see 29 to 34 Days
$4,331.68
Details
HOST_Wheat germ Human GST tag 10 μg Log in to see 9 Days
$405.71
Details
HOST_HEK-293 Cells Human Myc-DYKDDDDK Tag Validation with Western Blot 20 μg Log in to see 10 to 12 Days
$785.40
Details

BCS1L Proteins by Origin and Source

Origin Expressed in Conjugate
Human , , ,
, ,
Mouse (Murine) ,
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More Proteins for BCS1-Like (S. Cerevisiae) (BCS1L) Interaction Partners

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

  1. * 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.

  2. 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.

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

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

  5. This region encompasses the BCS1L gene.

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

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

  8. a function of BCS1L is to promote the maturation of complex III and the incorporation of the Rieske iron-sulfur protein (show UQCRFS1 Proteins) into the nascent complex. Defective BCS1L leads to the formation of a catalytically inactive, structurally unstable complex III.

  9. 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

  10. BCS1L stimulates the assembly of the LETM1 (show LETM1 Proteins) complex. BCS1L knockdown caused disassembly of the respiratory chains as well as LETM1 (show LETM1 Proteins) downregulation and induced distinct changes in mitochondrial morphology.

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

  1. 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.

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

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

  4. 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) Protein Profile

Protein Summary

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

  • mitochondrial chaperone BCS1 (AFUA_3G13000)
  • mitochondrial chaperone bcs1 (CC1G_00820)
  • mitochondrial chaperone bcs1 (CC1G_00842)
  • mitochondrial chaperone BCS1 (CC1G_01181)
  • mitochondrial chaperone BCS1 (CC1G_01608)
  • mitochondrial chaperone BCS1 (CC1G_11035)
  • mitochondrial chaperone BCS1 (CC1G_11036)
  • mitochondrial chaperone BCS1 (Bm1_30135)
  • mitochondrial ATPase (bcs1lA)
  • mitochondrial ATPase (bcs1lB)
  • BC1 (ubiquinol-cytochrome c reductase) synthesis-like (BCS1L)
  • BC1 (ubiquinol-cytochrome c reductase) synthesis-like (bcs1l)
  • BCS1-like (S. cerevisiae) (BCS1L)
  • BCS1-like (yeast) (Bcs1l)
  • 9130022O19Rik protein
  • BCS protein
  • BCS1 protein
  • BJS protein
  • DDBDRAFT_0184131 protein
  • DDBDRAFT_0188274 protein
  • DDBDRAFT_0266725 protein
  • DDBDRAFT_0266726 protein
  • DDB_0184131 protein
  • DDB_0188274 protein
  • DDB_0266725 protein
  • DDB_0266726 protein
  • FLNMS protein
  • GRACILE protein
  • h-BCS protein
  • Hs.6719 protein
  • MC3DN1 protein
  • PTD protein

Protein level used designations for BCS1L

mitochondrial chaperone BCS1 , mitochondrial chaperone bcs1 , BCS1-like protein , h-BCS1 , mitochondrial complex III assembly

GENE ID SPECIES
3512380 Aspergillus fumigatus Af293
6007738 Coprinopsis cinerea okayama7130
6007764 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
617 Homo sapiens
380366 Xenopus laevis
539713 Bos taurus
66821 Mus musculus
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