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BCS1L 产品

(BCS1-Like (BCS1L))

Categories

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. [provided by RefSeq, Mar 2012].

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Featured BCS1L Categories

BCS1L 抗体

High quality antibodies with extensive validation data.

BCS1L 蛋白

Proteins for various applications incl. WB, ELISA, IF etc.

Recommended BCS1L 抗体

Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Application WB, ELISA
Validations
  • (2)
  • (3)
Cat. No. ABIN560048
Quantity 100 μg
Datasheet Datasheet
Reactivity Human
Application WB, IHC, IF
Validations
  • (4)
Cat. No. ABIN6137520
Quantity 100 μL
Datasheet Datasheet
Reactivity Human, Mouse, Rat
Application IHC, IF
Validations
  • (3)
Cat. No. ABIN7263641
Quantity 200 μL
Datasheet Datasheet

Recommended BCS1L 蛋白

Product
Reactivity
Source
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Source Wheat germ
Validations
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Cat. No. ABIN7199684
Quantity 2 μg
Datasheet Datasheet
Reactivity Human
Source Wheat germ
Validations
  • (1)
Cat. No. ABIN1346607
Quantity 10 μg
Datasheet Datasheet
Reactivity Human
Source HEK-293 Cells
Validations
  • (1)
Cat. No. ABIN2715212
Quantity 20 μg
Datasheet Datasheet

Latest Publications for our BCS1L Products

Kotarsky, Karikoski, Mörgelin, Marjavaara, Bergman, Zhang, Smet, van Coster, Fellman: "Characterization of complex III deficiency and liver dysfunction in GRACILE syndrome caused by a BCS1L mutation." in: Mitochondrion, Vol. 10, Issue 5, pp. 497-509, (2010) (PubMed).

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, Vol. 9, Issue 5, pp. 299-305, (2009) (PubMed).

Synonyms and alternative names related to BCS1L

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

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
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