HIBCH Antibody

• Catalog number
  bs-15484R
• Price
  Please ask
• Size
  0.1ml

• Long name
  HIBCH Primary Polyclonal Antibody
• Also known as
  HIBCH PAb
• Category
  Primary Antibodies
• Conjugation
  Unconjugated
• Target Antigen
  HIBCH
• Specificity
  This is a highly specific antibody against HIBCH.
• 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 HIBCH
• Gene ID number
  26275
• 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
• Cross reactive species details
  Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
• Background of the target antigen
  HIBCH is a 386 amino acid protein belonging to the enoyl-CoA hydratase/isomerase family. Localizing to the mitochondria, HIBCH is highly expressed in liver and kidney, with lower levels found in heart, muscle and brain. HIBCH hydrolyzes HIBYL-CoA, a saline catabolite, and β-hydroxypropionyl-CoA, an intermediate in the minor pathway involved in the metabolism of proprionate. Existing as two alternatively spliced isoforms, the gene encoding HIBCH maps to human chromosome 2q32.2. Defects to this gene result in HIBCH deficiency (HIBCHD), known alternatively as deficiency of β-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. HIBCHD is characterized by the accumulation of methacrylyl-CoA, a highly reactive compound that undergoes addition reactions with free sulfhydryl groups. Phenotypic symptoms include early deterioration of neurological function, delayed motor skill development and hypotonia.
• 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
  3 hydroxyisobutyryl Coenzyme A hydrolase; 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial; 3-hydroxyisobutyryl-CoA hydrolase; 3-hydroxyisobutyryl-coenzyme A hydrolase; BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE; HIB CoA hydrolase; HIB-CoA hydrolase; HIBCH; HIBCH_HUMAN; HIBCoA hydrolase; HIBYL CoA H; HIBYL CoAH; HIBYL-CoA-H; HIBYLCoA H; HIBYLCoAH; mitochondrial; HIBCH_HUMAN.
• 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
  HIBCH  
• Gene symbol
  HIBCH
• Short name
  Anti-HIBCH
• Technique
  Antibody, antibodies against human proteins, antibodies for
• Host
  Rabbit (Oryctolagus cuniculus)
• Isotype
  Immunoglobulin G (IgG)
• Alternative name
  HIBCH Polyclonal Antibody
• Alternative technique
  antibodies

Gene info

• Identity
  HGNC:4908
• Gene
  HIBCH
• Long gene name
  3-hydroxyisobutyryl-CoA hydrolase
• Synonyms gene name
  • 3-hydroxyisobutyryl-Coenzyme A hydrolase
• GenBank acession
  • U66669
• Locus
  2q32.2
• Discovery year
  1999-12-07
• Entrez gene record
  26275
• Pubmed identfication
  • 8824301
• VEGA ID
  OTTHUMG00000132673

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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