HEXA/Beta hexosaminidase subunit alpha Antibody, Biotin Conjugated

• Catalog number
  bs-11726R-Biotin
• Price
  Please ask
• Size
  0.1ml

• Long name
  HEXA/Beta hexosaminidase subunit alpha Antibody, Biotin Conjugated
• Category
  Conjugated Primary Antibodies
• Conjugation
  Biotin
• Host Organism
  Rabbit (Oryctolagus cuniculus)
• Target Antigen
  HEXA/Beta hexosaminidase subunit alpha
• Specificity
  This is a highly specific antibody against HEXA/Beta hexosaminidase subunit alpha.
• Modification
  Unmodified
• Modification site
  None
• Clonality
  Polyclonal
• Clone
  Polyclonal antibody
• Concentration
  1ug per 1ul
• Source
  KLH conjugated synthetic peptide derived from human Beta hexosaminidase subunit alpha
• Tested applications
  WB, IHC-P
• Recommended dilutions
  WB(1:100-1000), IHC-P(1:100-500)
• Crossreactivity
  Human, Mouse, Rat
• Crossreactive species details
  Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
• Antigen background
  Hexosaminidase A (HEXA), also designated beta-Hexosaminidase A, is a trimer composed of one å chain, one ∫-A chain and one ∫-B chain and is found in the lysosomes of cells. HEXA, along with the cofactor CM2 activator protein, catalyzes the degradation of GM2 ganglioside and other molecules containing terminal N-acetyl hexosamines in the brain and other tissues. A mutation in the å subunit of hexosaminidase is the cause of Tay-Sachs disease (TSD), also known as GM2-gangliosidosis type I. TSD is a fatal autosomal recessive lysosomal storage disease of the central nervous system (CNS) caused by insufficient activity of the HEXA enzyme that results in a failure to process GM2 gangliosides. The accumulation of GM2 ganglioside in the absence of HEXA activity causes progressive destruction of the CNS.
• Purification method
  This antibody was purified via Protein A.
• Storage conditions
  Keep the antibody in an aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store refrigerated at 2 to 8 degrees Celcius for up to 1 year.
• Excitation Emission
  N/A
• Synonyms
  Beta hexosaminidase alpha chain precursor; Beta hexosaminidase subunit alpha; Beta N acetylhexosaminidase; Beta N acetylhexosaminidase subunit alpha; Beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; Hexa; HEXA_HUMAN; Hexosaminidase A alpha polypeptide; Hexosaminidase A alpha polypeptide; Hexosaminidase A; Hexosaminidase subunit A; MGC99608; N acetyl beta glucosaminidase; N acetyl beta glucosaminidase subunit alpha; N-acetyl-beta-glucosaminidase subunit alpha; TSD.
• Description
  The Anti-HEXA/Beta hexosaminidase subunit alpha is a α- or alpha protein sometimes glycoprotein present in blood.
• Properties
  If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24°C for long term storage and for short term at + 5°C. Biotin conjugates can be detected by horseradish peroxidase, alkaline phosphatase substrates or anti biotin conjugated antibodies. Avidin and Streptavidin bind to the small biotin and are couple to HRP or AP for ELISA. To break the streptavidin Biotin bond we suggest to use a 6 molar guanidine HCl solution with acidity of pH 1.6.
• Additional conjugation
  Biotinylated
• French translation
  anticorps

• Gene target
  HEXA/Beta   hexosaminidase   subunit   alpha   Conjugated  
• Gene symbol
  HEXA, HEXA-AS1
• Short name
  Anti-HEXA/Beta hexosaminidase subunit alpha
• Technique
  Antibody, antibodies against human proteins, antibodies for, antibody Conjugates
• Isotype
  Immunoglobulin G (IgG)
• Label
  Biotin
• Alternative name
  Anti-HEXA/Beta hexosaminidase subunit alpha PAb Biotin
• Alternative technique
  antibodies

Gene info

• Identity
  HGNC:4878
• Gene
  HEXA
• Long gene name
  hexosaminidase subunit alpha
• Synonyms gene name
  • hexosaminidase A (alpha polypeptide)
• Synonyms name
  • Tay Sachs disease
  • GM2 gangliosidosis
  • beta-hexosaminidase subunit alpha
• GenBank acession
  • M13520
• Locus
  15q23
• Discovery year
  2001-06-22
• Entrez gene record
  3073
• Pubmed identfication
  • 2952641
  • 3013851
• RefSeq identity
  • NM_000520
• Classification
  • Hexosaminidases
• VEGA ID
  OTTHUMG00000133445
• Locus Specific Databases
  • Hexosaminidase A; Tay-Sachs Disease
  • ALSOD, the Amyotrophic Lateral Sclerosis Online Genetic Database

Gene info

• Identity
  HGNC:25810
• Gene
  HEXA-AS1
• Long gene name
  HEXA antisense RNA 1
• Synonyms gene
  • C15orf34
• Synonyms gene name
  • chromosome 15 open reading frame 34
  • HEXA antisense RNA 1 (non-protein coding)
• Synonyms
  • FLJ13315
• Locus
  15q23
• Discovery year
  2005-10-20
• Entrez gene record
  80072
• Pubmed identfication
  • 12477932
• RefSeq identity
  • NR_027262
• Classification
  • Antisense RNAs
• VEGA ID
  OTTHUMG00000177184

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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