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

Name: Mouse Anti-BCS1L Recombinant Antibody (59)
SKU: CBMAB-AP607LY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

Application

ELISA, IF, IHC, WB

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.

More Infomation

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

Human	617
Mouse	66821

UniProt ID

Human	Q9Y276
Mouse	Q9CZP5

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

Baker, R. A., Priestley, J. R., Wilstermann, A. M., Reese, K. J., & Mark, P. R. (2019). Clinical spectrum of BCS1L Mitopathies and their underlying structural relationships. American Journal of Medical Genetics Part A, 179(3), 373-380.

Oláhová, M., Berti, C. C., Collier, J. J., Alston, C. L., Jameson, E., Jones, S. A., ... & Sayer, J. A. (2019). Molecular genetic investigations identify new clinical phenotypes associated with BCS1L-related mitochondrial disease. Human molecular genetics, 28(22), 3766-3776.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Antibody Pairs

BCS1L Matched Antibody Pair (134) (CAT#: APMAB-134LY)

Related Products

Mouse Anti-BCS1L Recombinant Antibody (CF049) (CAT#: CBMAB-FT425LY)

BCS1L Matched Antibody Pair (134) (CAT#: APMAB-134LY)

Isotype control

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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