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
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
- (1)
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
: "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).: "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:56205Protein 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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