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Rabbit Anti-BCS1L Antibody (CSB-PA897463ESR2HU)
Supplier: CUSABIO BIOTECH CO.
Mitochondrial chaperone BCS1 Antibody,h-BCS1 Antibody,BCS1-like protein Antibody,BCS1L Antibody,BCS1 AntibodyMore alternative names for the antibody
BCS 1 antibody|BCS 1L antibody|BCS antibody|BCS1 (yeast homolog) like antibody|BCS1 antibody|BCS1 like (S. cerevisiae) antibody|BCS1 like antibody|BCS1 like protein antibody|Bjornstad syndrome (pili torti and deafness) antibody|BJS antibody|FLNMS antibody|GRACILE antibody|h BCS antibody|H BCS1 antibody|Hs.6719 antibody|Mitochondrial chaperone BCS1 antibody|Mitochondrial complex III assembly antibody|PTD antibody
Anti-BCS1L antibody (ab102808)
Anti-BCS1L antibody (ab102808)
Recommended applications: ELISA, WB, IHC
Recommended dilution: User optimized
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Mitochondrial chaperone BCS1 protein (33-240AA)
ELISA, WB, IHC
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Protein function|| |
Chaperone necessary for the assembly of mitochondrial respiratory chain complex III. Plays an important role in the maintenance of mitochondrial tubular networks, respiratory chain assembly and formation of the LETM1 complex. .
|Protein tissue specificity|| |
|Involvement in disease|| |
GRACILE syndrome (GRACILE) [MIM:603358]: GRACILE stands for ‘growth retardation, aminoaciduria, cholestasis, iron overload, lactic acidosis, and early death’. It is a recessively inherited lethal disease characterized by fetal growth retardation, lactic acidosis, aminoaciduria, cholestasis, and abnormalities in iron metabolism. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Mitochondrial complex III deficiency, nuclear 1 (MC3DN1) [MIM:124000]: A disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance. . Note=The disease is caused by mutations affecting the gene represented in this entry.; Bjoernstad syndrome (BJS) [MIM:262000]: An autosomal recessive disease characterized by congenital sensorineural hearing loss and twisted hairs (pili torti). Pili torti is a condition in which the hair shafts are flattened at irregular intervals and twisted 180 degrees from the normal axis, making the hair extremely brittle. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the AAA ATPase family. BCS1 subfamily.
|Protein cellular localization|| |
Mitochondrion inner membrane ; Single-pass membrane protein .
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CUSABIO BIOTECH CO.
|Product type|| |
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