Anti-HEXB Antibody [HEXB/7762] - BSA and Azide free

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Descriere

Specificații

Brand

Antibodies

Mărime

100µg

Țintă

HEXB

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

63 kDa

Light chain

kappa

Clonă

HEXB/7762

Imunogen

Recombinant fragment human HEXB protein. The exact sequence is proprietary.

Specificitate

Hexosaminidase B (HEXB), also designated subunit. These mutations disrupt the activity of HEXB and HEXA, which prevents the breakdown of GM2 ganglioside, a fatty material found in the brain, therby rendering both the HEXA and HEXB enzymes deficient. SD is a rare autosomal recessive disorder characterized by an accumulation of GM2 ganglioside, which causes progressive destruction of the central nervous system. Sandhoff disease is similar to Tay-Sachs disease, which is caused by mutations in the HEXA gene, although SD is more severe.

Storage

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

Documentație

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