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Mouse Anti-BCS1L Recombinant Antibody (59) (CBMAB-AP607LY)


See all BCS1L antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse
Clone
59
Antibody Isotype
IgG2a
Application
ELISA, IF, IHC, WB

Basic Information

Immunogen
Fusion protein of BCS1L.
Host Species
Mouse
Specificity
Human, Mouse
Antibody Isotype
IgG2a
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, 50% glycerol, pH 7.3
Preservative
0.02% sodium azide
Concentration
Batch dependent
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
BCS1 Homolog, Ubiquinol-Cytochrome C Reductase Complex Chaperone
Introduction
BCYRN1P1 (Brain Cytoplasmic RNA 1, Pseudogene 1) is a Pseudogene.
Entrez Gene ID
Human617
Mouse66821
UniProt ID
HumanQ9Y276
MouseQ9CZP5
Alternative Names
BCS1 Homolog, Ubiquinol-Cytochrome C Reductase Complex Chaperone; BC1 (Ubiquinol-Cytochrome C Reductase) Synthesis-Like; BCS1-Like Protein; H-BCS1; BCS1; Mitochondrial Complex III Assembly; Mitochondrial Chaperone BCS1; BCS1 (Yeast Homolog)-Like; BCS1-Like (S. Cerevisiae); Bjornstad Syndrome; BCS1-Like (Yeast);
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.
Biological Process
Mitochondrial cytochrome c oxidase assembly Source: UniProtKB
Mitochondrial respiratory chain complex I assembly Source: UniProtKB
Mitochondrial respiratory chain complex III assembly Source: UniProtKB
Mitochondrion organization Source: UniProtKB
Protein insertion into mitochondrial inner membrane from matrix Source: GO_Central
Cellular Location
Mitochondrion inner membrane
Involvement in disease
GRACILE syndrome (GRACILE): 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.
Mitochondrial complex III deficiency, nuclear 1 (MC3DN1): 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.
Bjoernstad syndrome (BJS): 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.
Topology
Mitochondrial intermembrane: 2-15 aa
Helical: 16-32 aa
Mitochondrial matrix: 33-419 aa
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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