Anti-BCS1L Antibody

$99.00$319.00

Reactivity: Human,Mouse
Applications: ELISA,WB,IHC
Conjugation: Various
Supplier: CUSABIO BIOTECH CO.

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Rabbit Anti-BCS1L Antibody (CSB-PA897463ESR1HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

Mitochondrial chaperone BCS1 Antibody,h-BCS1 Antibody,BCS1-like protein Antibody,BCS1L Antibody,BCS1 Antibody

More 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)
Close sc-134280|

Recommended applications: ELISA, WB, IHC

Recommended dilution: User optimized

Recommended protocols: check protocols

Name

Anti-BCS1L Antibody

Catalogue No.

CSB-PA897463ESR1HU

Reactivity

Human, Mouse

Immunogen

Recombinant human Mitochondrial chaperone BCS1 protein (33-240AA)

Host

Rabbit

Applications

ELISA, WB, IHC

Recommended dilution

User optimized

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

47 kDa

Purification

Antigen Affinity Purified

Storage

Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.

Database links

Entrez Gene:617( Human), Entrez Gene:66821( Mouse), Omim:603647( Human), SwissProt:Q9Y276( Human), SwissProt:Q9CZP5( Mouse), Unigene:471401( Human)

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

Ubiquitous. .

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 .

Research area

All research areas>Synthesis and Degradation>BCS1L
(View all antibody categories related to Synthesis and Degradation)

Note

AntibodyPlus provides antibody trial sample for your own antibody validation and collects antibody reviews.

Supplier

CUSABIO BIOTECH CO.

Product type

Primary antibody

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