GLA/alpha-Galactosidase A Protein, Mouse (HEK293, His)

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GLA/alpha-Galactosidase A Protein is responsible for the breakdown of alpha-galactoside in the lysosome. GLA/alpha-Galactosidase A Protein participates in the negative regulation of nitric oxide biosynthesis and nitric oxide synthase activity. A deficiency of GLA/alpha-Galactosidase A Protein causes Fabry disease. GLA/alpha-Galactosidase A Protein, Mouse (HEK293, His) is the recombinant mouse-derived GLA/alpha-Galactosidase A protein, expressed by HEK293 , with C-His labeled tag.

For research use only. We do not sell to patients.
  • Species: Mouse
  • Source: HEK293
  • Storage:
    Stored at -20°C for 2 years from date of receipt. After reconstitution, it is stable at 4°C for 1 week or -20°C for longer (with carrier protein). It is recommended to freeze aliquots at -20°C or -80°C for extended storage.
  • Biological Activity
  • Technical Parameters
  • Product Properties
  • Documentation
  • Help & FAQs

Biological Activity

Description

GLA/alpha-Galactosidase A Protein is responsible for the breakdown of alpha-galactoside in the lysosome. GLA/alpha-Galactosidase A Protein participates in the negative regulation of nitric oxide biosynthesis and nitric oxide synthase activity. A deficiency of GLA/alpha-Galactosidase A Protein causes Fabry disease. GLA/alpha-Galactosidase A Protein, Mouse (HEK293, His) is the recombinant mouse-derived GLA/alpha-Galactosidase A protein, expressed by HEK293 , with C-His labeled tag.

Background

Alpha-galactosidase A is responsible for the breakdown of alpha-galactoside in lysosomes and is homologous to human galactosidase (GLA). GLA is involved in the negative regulation of nitric oxide biosynthesis and nitric oxide synthase activity. GLA acts upstream or internally in the catabolic process of glycosylceramide. Mice deficient in alpha-galactosidase A exhibit age-dependent and distinct sensory system deficits. This is a lysosome storage disorder characterized by the accumulation of alpha-galactosylated substrates in tissues. Alpha-galactosidase A is an active target of clinical research: there are currently two treatment options for Fabry disease, recombinant enzyme replacement therapy and drug companion therapy. Tlr-dependent alpha-Gal-A negative regulation is the mechanistic link between pathogen recognition and autolipid antigen induction in NKT cells[1][2][3][4].

Verified Bioactivity

Measured by its ability to hydrolyze 4-methylumbelliferyl-alpha-D-galactopyranoside. The specific activity is 833.54 pmoL/min/µg, as measured under the described conditions.

MCE Validation Data

  • Purity - SDS-PAGE

    Purity - SDS-PAGE

    ≥ 95%, as determined by reducing SDS-PAGE.

Technical Parameters

  • Species Mouse
  • Source HEK293
  • Tag C-His
  • Accession
  • Gene ID
  • Molecular Construction
    • N-term
    • GLA (L34-R421)
      Accession # Q8BGZ6
    • His
    • C-term
  • Protein Length

    Full Length of Mature Protein

  • Synonyms

    GLA; Alpha-D-Galactoside Galactohydrolase 1; Galactosidase Alpha; Alpha-D-Galactoside Galactohydrolase; GALA; Galactosidase, Alpha; Galactosylgalactosylglucosylceramidase GLA; Alpha-Galactosidase; Alpha-D-Galactosidase A; Agalsidase Alfa; Alpha-Galactosid

  • AA Sequence

    LDNGLARTPTMGWLHWERFMCNLDCQEEPDACISEQLFMQMAELMVSDGWRDAGYDYLCIDDCWMAPERDSKGRLQADPQRFPSGIKHLANYVHSKGLKLGIYADVGNKTCAGFPGSFGSYDIDAQTFADWGVDLLKFDGCHCDSVVSLENGYKYMALALNRTGRSIVYSCEWPLYLRPFHKPNYTDIQYYCNHWRNFDDVYDSWESIKNILSWTVVYQKEIVEVAGPGSWNDPDMLVIGNFGLSWDQQVTQMALWAIMAAPLLMSNDLRQISSQAKALLQNKDVIAINQDPLGKQGYCFRKENHIEVWERPLSNLAWAVAVRNLQEIGGPCPYTIQISSLGRGLACNPGCIITQLLPEKVHLGFYEWTLTLKTRVNPSGTVLFRLER

  • Molecular Weight

    Approximately 46-52 kDa, based on SDS-PAGE under reducing conditions, due to the glycosylation.

  • Purity

    ≥ 95%, as determined by reducing SDS-PAGE.

Product Properties

Appearance

Lyophilized powder.

Formulation

Lyophilized from a 0.22 μm filtered solution of PBS, pH 7.4.

Endotoxin Level

<1 EU/μg, determined by LAL method.

Reconstitution

It is not recommended to reconstitute to a concentration less than 100 μg/mL in ddH2O. For long term storage it is recommended to add a carrier protein (0.1% BSA, 5% HSA, 10% FBS or 5% Trehalose).

Storage & Stability

Stored at -20°C for 2 years from date of receipt. After reconstitution, it is stable at 4°C for 1 week or -20°C for longer (with carrier protein). It is recommended to freeze aliquots at -20°C or -80°C for extended storage.

Shipping

Room temperature in continental US; may vary elsewhere.

Calculators

Reconstitution Calculator

Volume (to add to vial) = Mass (in vial) ÷ Desired Reconstitution Concentration

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Desired Reconstitution Concentration Desired Reconstitution Concentration
Dilution Calculator

Concentration (start) × Volume (start) = Concentration (final) × Volume (final)

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The Specific Activity Calculator Equation
  • Specific Activity (Unit/mg)
  • Biological Activity (ED50)

Specific Activity (Unit/mg) = 106 ÷ Biological Activity (ED50)

Specific Activity (Unit/mg) Specific Activity (Unit/mg)
Unit/mg
= 106 ÷
Biological Activity (ED50) Biological Activity (ED50)
106 ÷
ng/mL
MOQ
Minimum order quantity
100 mg

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