Mouse Hexosaminidase A Alpha (HEXa) Protein

Este producto es parte de HEXA - Beta-Hexosaminidase Subunit Alpha
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741€ (50 µg)

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935106861
info@markelab.com
name
Mouse Hexosaminidase A Alpha (HEXa) Protein
category
Proteins and Peptides
provider
Abbexa
reference
abx651347
tested applications
WB, SDS-PAGE

Description

Hexosaminidase A Alpha (HEXa) Protein (Active) is an active protein from Mouse.

Documents del producto

Instrucciones
Data sheet
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Product specifications

Category
Proteins and Peptides
Immunogen Target
Hexosaminidase A Alpha (HEXa)
Host
E. coli
Origin
Mouse
Conjugation
Unconjugated
Observed MW
Molecular Weight: Calculated MW: 28.2 kDa  Observed MW: 28 kDa

Concentration: Prior to lyophilization: 200 µg/ml

Sequence Fragment: Leu319-Thr528

Tag: N-terminal His tag
Purity
> 97%
Size 1
50 µg
Size 2
100 µg
Size 3
200 µg
Size 4
500 µg
Size 5
1 mg
Form
Lyophilized To keep the original salt concentration, we recommend reconstituting to the original concentration prior to lyophilization (see Concentration) in ddH2O. If a lower concentration is required, dilute in 20 mM Tris, 150 mM NaCl, pH 8.0. If a higher concentration is required, the product can be reconstituted directly in 20 mM Tris, 150 mM NaCl, pH 8.0, though please note that this will change the overall salt concentration. The stock concentration should be between 0.1-1.0 mg/ml. Do not vortex.
Tested Applications
WB, SDS-PAGE
Buffer
Prior to lyophilization: 20 mM Tris, 150 mM NaCl, pH 8.0, containing 1 mM EDTA, 1 mM DTT, 0.01% Sarcosyl, 5% Trehalose and Proclin-300.
Availability
Shipped within 5-7 working days.
Storage
Store at 2-8 °C for up to one month. Store at -80 °C for up to one year. Avoid repeated freeze/thaw cycles.
Dry Ice
No
UniProt ID
P29416
Alias
N-acetyl-beta-glucosaminidase subunit alpha,TSD
Background
Protein HEXA
Status
RUO
Note
This product is for research use only.   Not for human consumption, cosmetic, therapeutic or diagnostic use.

Descripción

HEXA is the alpha subunit of the beta-hexosaminidase enzyme, a lysosomal hydrolase that catalyzes the breakdown of GM2 gangliosides into GM3 in the process of glycosphingolipid degradation The enzyme functions as a heterodimer composed of alpha and beta subunits, encoded by the HEXA and HEXB genes respectively Mutations in HEXA lead to Tay-Sachs disease, an autosomal recessive lysosomal storage disorder characterized by the accumulation of GM2 gangliosides in neuronal cells, resulting in neurodegeneration, progressive motor weakness, and developmental delay HEXA is highly active in neuronal tissues where glycosphingolipid metabolism is critical for normal brain function The absence or dysfunction of HEXA causes a loss of enzymatic activity leading to cellular toxicity and apoptosis Current therapeutic approaches include enzyme replacement therapy, substrate reduction therapy, and gene therapy to restore HEXA activity and slow disease progression

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