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

BCS1L encodes a homolog of the S. Zusätzlich bieten wir Ihnen BCS1-Like (S. Cerevisiae) Proteine (8) und BCS1-Like (S. Cerevisiae) Kits (5) und viele weitere Produktgruppen zu diesem Protein an.

Alle Antikörper anzeigen Gen GeneID UniProt
BCS1L 617 Q9Y276
BCS1L 66821 Q9CZP5
BCS1L    
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Top anti-BCS1-Like (S. Cerevisiae) Antikörper auf antikoerper-online.de

Showing 10 out of 19 products:

Katalog Nr. Reaktivität Wirt Konjugat Applikation Bilder Menge Anbieter Lieferzeit Preis Details
Human Maus Unkonjugiert ELISA, WB Western Blot analysis of BCS1L expression in transfected 293T cell line by BCS1L monoclonal antibody (M01), clone 5F3.<br><br>Lane 1: BCS1L transfected lysate(47.534 KDa).<br>Lane 2: Non-transfected lysate.<br> 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 Anmelden zum Anzeigen 11 bis 12 Tage
$430.00
Details
Human Maus Unkonjugiert 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. 0.1 mg Anmelden zum Anzeigen 8 bis 11 Tage
$450.00
Details
Human Kaninchen Unkonjugiert IHC, WB Western blot analysis of extracts of various cell lines, using BCS1L antibody. Immunohistochemistry of paraffin-embedded human gastric using BCS1L antibody at dilution of 1:100 (x40 lens). 100 μL Anmelden zum Anzeigen 16 Days
$323.13
Details
Human Maus Unkonjugiert WB Western Blot analysis of BCS1L expression in transfected 293T cell line by BCS1L MaxPab polyclonal antibody.<br><br>Lane 1: BCS1L transfected lysate(46.09 KDa).<br>Lane 2: Non-transfected lysate.<br> 50 μL Anmelden zum Anzeigen 11 bis 12 Tage
$430.00
Details
Human Kaninchen Unkonjugiert IHC, IHC (p) Immunohistochemistry: BCS1L Antibody [NBP1-88678] - Staining of human heart muscle shows strong cytoplasmic positivity in cardiac myocytes. 0.1 mL Anmelden zum Anzeigen 10 bis 13 Tage
$439.69
Details
Human Kaninchen Unkonjugiert IHC, IHC (p), WB Western Blot: BCS1L Antibody [NBP1-88677] - Lane 1: Marker [kDa] 250, 130, 95, 72, 55, 36, 28, 17, 10.Lane 2: Human cell line RT-4.Lane 3: Human cell line U-251MG sp Immunohistochemistry-Paraffin: BCS1L Antibody [NBP1-88677] - Staining of human liver shows strong cytoplasmic positivity in hepatocytes. 0.1 mL Anmelden zum Anzeigen 10 bis 13 Tage
$439.69
Details
Human Maus Unkonjugiert ELISA, WB   100 μg Anmelden zum Anzeigen 7 bis 9 Tage
$537.17
Details
Human Kaninchen Unkonjugiert WB Western Blot analysis of BCS1L expression in transfected 293T cell line by BCS1L MaxPab polyclonal antibody.<br><br>Lane 1: BCS1L transfected lysate(47.50 KDa).<br>Lane 2: Non-transfected lysate.<br> BCS1L MaxPab rabbit polyclonal antibody. Western Blot analysis of BCS1L expression in mouse spleen. 100 μg Anmelden zum Anzeigen 11 bis 12 Tage
$418.57
Details
Human Kaninchen Unkonjugiert WB   100 μg Anmelden zum Anzeigen 7 bis 9 Tage
$903.83
Details
Human Kaninchen Unkonjugiert IHC, ELISA, WB   50 μL Anmelden zum Anzeigen 13 bis 16 Tage
$313.50
Details

BCS1L Antikörper nach Reaktivität, Anwendung, Klonalität and Konjugat

Attribute Applikationen Wirt Klonalität
Human ,
,
,
Mouse (Murine)


Rat (Rattus)


Am meisten referenzierte anti-BCS1-Like (S. Cerevisiae) Antikörper

  1. Human Monoclonal BCS1L Primary Antibody für 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. in Mitochondrion 2009 (PubMed)
    Show all 2 Pubmed References

Weitere Antikörper gegen BCS1-Like (S. Cerevisiae) Interaktionspartner

Human BCS1-Like (S. Cerevisiae) (BCS1L) Interaktionspartner

  1. 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

  2. * 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.

  3. 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.

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

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

  6. This region encompasses the BCS1L gene.

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

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

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

  10. 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

Mouse (Murine) BCS1-Like (S. Cerevisiae) (BCS1L) Interaktionspartner

  1. COX7A2L (zeige COX7A2L Antikörper)/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) Antigen-Profil

Protein Überblick

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.

Genbezeichner und Symbole assoziert mit BCS1L

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

Bezeichner auf Proteinebene für BCS1L

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

GENE ID SPEZIES
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
Ausgewählte Anbieter für anti-BCS1-Like (S. Cerevisiae) (BCS1L) Antikörper
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