GBE1 Antibody

• Catalog number
  bs-13300R
• Price
  Please ask
• Size
  0.1ml

• Long name
  GBE1 Primary Polyclonal Antibody
• Also known as
  GBE1 PAb
• Category
  Primary Antibodies
• Conjugation
  Unconjugated
• Target Antigen
  GBE1
• Specificity
  This is a highly specific antibody against GBE1.
• Modification s
  None
• Modification site s
  Unmodified antibody
• Clonality
  Polyclonal antibody
• Clone number
  Polyclonal antibody
• Concentration
  1ug per 1ul
• Source
  This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human GBE1
• Gene ID number
  2632
• Tested Applications
  WB, IHC-P, IF(IHC-P)
• Recommended dilutions
  WB(1:100-1000), IHC-P(1:100-500), IF(IHC-P)(1:50-200)
• Cross reactivity
  Human, Mouse, Rat
• Cross reactive species details
  Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
• Background of the target antigen
  GBE1 is a 702 amino acid protein that is expressed at high levels in muscle and liver and is involved in glycogen biosynthesis. Existing as a monomer, GBE1 catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on a neighboring glycogen chain and, via this catalytic activity, plays an essential role in glycogen accumulation. Defects in the gene encoding GBE1 are the cause of glycogen storage disease type 4 (GSD4) and adult polyglucosan body disease (APBD), the first of which is a metabolic disorder that is associated with the accumulation of polysaccharides and is characterized by liver disease during childhood. Unlike GSD4, APBD is a late-onset disorder that affects the central and peripheral nervous systems and is characterized by cognitive impairment, pyramidal tetraparesis and peripheral neuropathy.
• Purification method
  This antibody was purified via Protein A.
• Storage conditions
  Keep the antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store at -20°C for up to 1 year.
• Synonym names
  1,4 alpha glucan branching enzyme; 4-alpha-glucan-branching enzyme; amylo 1,4 to 1,6 transglucosidase; amylo 1,4 to 1,6 transglycosylase; Andersen disease; Brancher enzyme; GBE 1; GBE; GBE1; gGlucan 1,4 alpha , branching enzyme 1; GLGB_HUMAN; Glucan 1,4 alpha branching enzyme; Glycogen branching enzyme; Glycogen storage disease type IV; Glycogen-branching enzyme; OTTHUMP00000213788; OTTHUMP00000213833.
• Properties
  If you buy Antibodies supplied by Bioss Primary Unconjugated Antibodies they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
• French translation
  anticorps

• Gene target
  GBE1  
• Gene symbol
  GBE1
• Short name
  Anti-GBE1
• Technique
  Antibody, antibodies against human proteins, antibodies for
• Host
  Rabbit (Oryctolagus cuniculus)
• Isotype
  Immunoglobulin G (IgG)
• Alternative name
  GBE1 Polyclonal Antibody
• Alternative technique
  antibodies

Gene info

• Identity
  HGNC:4180
• Gene
  GBE1
• Long gene name
  1,4-alpha-glucan branching enzyme 1
• Synonyms gene name
  • glucan (1,4-alpha-), branching enzyme 1
• Synonyms name
  • glycogen branching enzyme
  • Andersen disease
  • glycogen storage disease type IV
• Locus
  3p12.2
• Discovery year
  1993-06-21
• Entrez gene record
  2632
• Pubmed identfication
  • 8463281
• VEGA ID
  OTTHUMG00000158978

MeSH Data

• Name
  Blotting, Western
• Concept
  Scope note: Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
• Tree numbers
  • E05.196.401.143
  • E05.301.300.096
  • E05.478.566.320.200
  • E05.601.262
  • E05.601.470.320.200
• Qualifiers
  ethics, trends, veterinary, history, classification, economics, instrumentation, methods, standards, statistics & numerical data

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