BCS1L Antibody 0 Publications

Mouse Monoclonal| Catalog number: 60212-1-Ig |CloneNo.: 3G6H1

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Freight/Packing

Con: 129 μg/150 μl

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Species specificity:
human, mouse

Positive WB detected in:
Colo320 cells, COLO 320 cells

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

Positive IF detected in:
MCF-7

Recommended dilution:
WB : 1:500-1:2000
IHC : 1:20-1:200
IF : 1:20-1:200

Product Information


Source:
Mouse

Purification method:
Protein G purification

Isotype:
IgG1

Storage:
PBS with 0.02% 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:
47KD

GenBank accession number:

Gene ID (NCBI):

Gene symbol
BCS1L

Synonyms
BCS1L, h BCS1, h BCS, GRACILE, FLNMS
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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