Anti-SDHA Antibody [SDHA/7491] - BSA and Azide free

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Descriere

Specificații

Brand

Antibodies

Mărime

100µg

Țintă

SDHA

Applications

IHC-P

Reactivity

Human

Formulation

Supplied in 10mM Phosphate Buffered Saline; without Sodium Azide and carrier free.

Conjugate

Unconjugated

Host

Mouse

Clonality

Monoclonal

Product form

Liquid

Concentration

1 mg/ml

Purification

Protein A/G chromatography.

Isotype

IgG2

Recommended dilutions

IHC: 1-2 µg/ml

Molecular weight

70 kDa

Light chain

kappa

Clonă

SDHA/7491

Imunogen

Recombinant fragment (around Amino Acids 450-650) of human SDHA protein. The exact sequence is proprietary.

Specificitate

In aerobic respiration reactions, succinate dehydrogenase (SDH) catalyzes the oxidation of succinate and ubiquinone to fumarate and ubiquinol. Four subunits comprise the SDH protein complex: a flavochrome subunit (SDHA), an iron-sulfur protein (SDHB) and two membrane-bound subunits (SDHC and SDHD) anchored to the inner mitochondrial membrane. Mutations to these subunits cause mitochondrial dysfunction, corresponding to several distinct disorders. Mutations in the membrane bound components may cause hereditary paraganglioma, while SDHA mutations are associated with juvenile encephalopathy as well as Leigh syndrome, a severe neurological disorder. Inactivating mutations in SDHB correlate with inherited, but not necessarily sporadic, cases of pheochromocytoma.

Storage

Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.

Documentație

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