Hexosaminidase A antibody (AA 135-179)
Quick Overview for Hexosaminidase A antibody (AA 135-179) (ABIN7997485)
Target
See all Hexosaminidase A (HEXA) AntibodiesReactivity
Host
Clonality
Conjugate
Application
Grade
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Binding Specificity
- AA 135-179
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Purpose
- Anti-HEXA Antibody
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Cross-Reactivity (Details)
- No cross-reactivity with other proteins.
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Characteristics
- Anti-HEXA Antibody. Tested in ELISA, IF, ICC, WB applications. This antibody reacts with Human, Mouse, Rat.
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Purification
- Immunogen affinity purified.
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Immunogen
- E.coli-derived human HEXA recombinant protein (Position: A135-H179).
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Isotype
- IgG
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Application Notes
- Western blot, 0.1-0.25 μg/mL, Human, Mouse, Rat Immunocytochemistry/Immunofluorescence, 5 μg/mL, Human ELISA, 0.1-0.5 μg/mL, -
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Restrictions
- For Research Use only
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Format
- Lyophilized
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Reconstitution
- Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
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Concentration
- 500 μg/mL
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Buffer
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
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Storage
- 4 °C,-20 °C
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Storage Comment
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At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing. -
Expiry Date
- 12 months
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- Hexosaminidase A (HEXA)
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Alternative Name
- HEXA
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Background
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Background: HEXA, hexosaminidase A (alpha polypeptide), is an enzyme that in humans is encoded by the HEXA gene. Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines. The HEXA gene encodes the alpha subunit of hexosaminidase A , a lysosomal enzyme involved in the breakdown of gangliosides. The HEXA gene is mapped on 15q23. Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit is able to hydrolyze GM2 gangliosides. The alpha subunit contains a key residue, Arg-424, which is essential for binding the N-acetyl-neuramanic residue of GM2 gangliosides. Chimeric constructs were expressed in HeLa cells and selected constructs were produced in the baculovirus expression system to determine their ability to degrade GM2 ganglioside in the presence of GM2 activator protein. Their results allowed them to define 2 noncontiguous sequences in the alpha subunit (amino acids 1-191 and 403-529) which, when substituted into analogous positions in the beta subunit, conferred activity against the sulfated substrate.
Gene Full Name: hexosaminidase subunit alpha
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Molecular Weight
- 60 kDa
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Gene ID
- 3073
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UniProt
- P06865
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Pathways
- Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
Target
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