BCS1L Antibody 5 Publications

Rabbit Polyclonal| Catalog number: 10175-2-AP

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Freight/Packing

Con: 50 μg/150 μl

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Species specificity:
human, mouse, rat

Positive WB detected in:
A549 cells, HEK-293 cells, HeLa cells, human brain tissue, mouse colon tissue, mouse kidney tissue, mouse liver tissue, mouse skeletal muscle tissue, mouse small intestine tissue, rat liver tissue

Positive IHC detected in:
human gliomas tissue, human kidney tissue

Positive IF detected in:
A549 cells

Recommended dilution:
WB : 1:500-1:1000
IHC : 1:20-1:200
IF : 1:20-1:200

Product Information


Source:
Rabbit

Purification method:
Antigen affinity purification

Isotype:
IgG

Storage:
PBS with 0.1% sodium azide and 50% glycerol pH 7.3. Store at -20oC. Aliquoting is unnecessary for -20oC storage.

Immunogen Information


Full name:
BCS1-like (yeast)

Calculated molecular weight:
48 kDa

Observed molecular weight:
50-55 kDa

GenBank accession number:

Gene ID (NCBI):

Gene symbol
BCS1L

Synonyms
BCS1L, BJS, FLNMS, GRACILE, h BCS, h BCS1, Hs.6719, Mitochondrial chaperone BCS1, PTD
Background

Human BCS1-like (BCS1L), a mitochondrial inner-membrane protein, is a chaperone necessary for the assembly of mitochondrial respiratory chain complex III. Recently studies indicated that the mutations in this protein may cause the GRACILE (growth retardation, aminoaciduria, cholestasis, iron overload, lactacidosis, and early death) syndrome, a recessively inherited lethal disease characterized by fetal growth retardation, lactic acidosis, aminoaciduria, cholestasis, and abnormalities in iron metabolism.


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