Product Datasheet  
Trifunctional enzyme subunit beta, mitochondrial Polyclonal Antibody  
Catalog Number: 42383  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot#: P55084
  • Form of Antibody：  
  • Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • Storage：  
  • Store at -20˚C
  • Immunogen：  
  • Recombinant human Trifunctional enzyme subunit beta, mitochondrial protein
  • reactivity：  
  • Hu
  • appl_detail：  

  • Immunohistochemistry: 1:20 - 1:200
  • other_names：  
  • P55084
  • Purification：  
  • Caprylic Acid Ammonium Sulfate Precipitation purified
  • Specificity：  
  • The antibody detects endogenous level of total Trifunctional enzyme subunit beta, mitochondrial polyclonal antibody.
  • Applications：  
  • IHC
  • Background：  
  • Defects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all three enzyme activities of the TFP complex.



 
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