BCS1L Antibody (SS-M13): sc-134280

BCS1L Antibody (SS-M13) is a mouse monoclonal IgG1 kappa light chain antibody that detects BCS1L protein of human origin by western blotting (WB), immunoprecipitation (IP), and enzyme-linked immunosorbent assay (ELISA). Anti-BCS1L antibody (SS-M13) is available as the non-conjugated form. BCS1L (SS-M13) antibody recognizes a protein that plays a crucial role in mitochondrial function as a chaperone necessary for the assembly of mitochondrial respiratory chain complex III, which is essential for ATP production through oxidative phosphorylation. Dysfunction in BCS1L can lead to severe mitochondrial disorders, including GRACILE syndrome, characterized by growth retardation, aminoaciduria, cholestasis, iron overload, and early death, as well as Björnstad syndrome, which is associated with sensorineural hearing loss. Mutations in BCS1L disrupt the assembly of complex III, leading to reduced activity of the mitochondrial electron transport chain and increased production of reactive oxygen species, which are linked to various clinical manifestations of mitochondrial diseases. BCS1L monoclonal antibody (SS-M13) accurately detects this protein, which is vital for understanding the underlying mechanisms of these disorders and for developing potential therapeutic strategies.

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BCS1L Antibody (SS-M13) References:

1. GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L.  |  Visapää, I., et al. 2002. Am J Hum Genet. 71: 863-76. PMID: 12215968
2. The GRACILE syndrome, a neonatal lethal metabolic disorder with iron overload.  |  Fellman, V. 2002. Blood Cells Mol Dis. 29: 444-50. PMID: 12547234
3. BCS1L is expressed in critical regions for neural development during ontogenesis in mice.  |  Kotarsky, H., et al. 2007. Gene Expr Patterns. 7: 266-73. PMID: 17049929
4. Missense mutations in the BCS1L gene as a cause of the Björnstad syndrome.  |  Hinson, JT., et al. 2007. N Engl J Med. 356: 809-19. PMID: 17314340
5. Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy.  |  Fernandez-Vizarra, E., et al. 2007. Hum Mol Genet. 16: 1241-52. PMID: 17403714
6. Mitochondrial hepatopathies: advances in genetics and pathogenesis.  |  Lee, WS. and Sokol, RJ. 2007. Hepatology. 45: 1555-65. PMID: 17538929
7. Liver disease in mitochondrial disorders.  |  Lee, WS. and Sokol, RJ. 2007. Semin Liver Dis. 27: 259-73. PMID: 17682973
8. Screening of BCS1L mutations in severe neonatal disorders suspicious for mitochondrial cause.  |  Fellman, V., et al. 2008. J Hum Genet. 53: 554-558. PMID: 18386115
9. Infantile mitochondrial encephalomyopathy with unusual phenotype caused by a novel BCS1L mutation in an isolated complex III-deficient patient.  |  Blázquez, A., et al. 2009. Neuromuscul Disord. 19: 143-6. PMID: 19162478

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-BCS1L Antibody (SS-M13) has 1 citations in a variety of scientific publications.

Primary antibodies like Anti-BCS1L Antibody (SS-M13) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

BCS1L Antibody (SS-M13) Specifications

Alternate Product Name: BCS1L

Product Clone Name: SS-M13

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG₁ kappa light chain

Amino Acids: 320-419

Epitope Target: BCS1L

Epitope Species: human

Product Reactivity/Detection: BCS1L

Species Reactivity/Detection: human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, ELISA

Vialing Information: Each vial contains 100 µg IgG₁ kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 1 citations in various scientific publications

Available Conjugates/Forms for BCS1L Antibody (SS-M13): N/A