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WB analysis of mouse liver(lane 1), kidney(lane 2) tissue lysate (35ug/lane) using GTX81126 EHHADH antibody, C-term.
WB analysis of mouse liver(lane 1), kidney(lane 2) tissue lysate (35ug/lane) using GTX81126 EHHADH antibody, C-term.
WB analysis of mouse liver(lane 1), kidney(lane 2) tissue lysate (35ug/lane) using GTX81126 EHHADH antibody, C-term.

EHHADH antibody, C-term

GTX81126
GeneTex
ApplicationsFlow Cytometry, Western Blot, ImmunoHistoChemistry, ImmunoHistoChemistry Paraffin
Product group Antibodies
TargetEHHADH
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Overview

  • Supplier
    GeneTex
  • Product Name
    EHHADH antibody, C-term
  • Delivery Days Customer
    9
  • Application Supplier Note
    WB: 1:1000. IHC-P: 1:10-1:50. FACS: 1:10-1:50. *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.
  • Applications
    Flow Cytometry, Western Blot, ImmunoHistoChemistry, ImmunoHistoChemistry Paraffin
  • Certification
    Research Use Only
  • Clonality
    Polyclonal
  • Conjugate
    Unconjugated
  • Gene ID1962
  • Target name
    EHHADH
  • Target description
    enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase
  • Target synonyms
    ECHD, FRTS3, L-PBE, LBFP, LBP, MFE1, PBFE, peroxisomal bifunctional enzyme, 3,2-trans-enoyl-CoA isomerase, L-3-hydroxyacyl-CoA dehydrogenase, L-bifunctional protein, peroxisomal, PBE, enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase, enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase, multifunctional enzyme 1, peroxisomal enoyl-CoA hydratase
  • Host
    Rabbit
  • Isotype
    IgG
  • Protein IDQ08426
  • Protein Name
    Peroxisomal bifunctional enzyme
  • Scientific Description
    The protein encoded by this gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. Defects in this gene are a cause of peroxisomal disorders such as Zellweger syndrome. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]
  • Storage Instruction
    -20°C or -80°C,2°C to 8°C
  • UNSPSC
    12352203

References

  • Ranea-Robles P, Portman K, Bender A, et al. Peroxisomal L-bifunctional protein (EHHADH) deficiency causes male-specific kidney hypertrophy and proximal tubular injury in mice. Kidney360. 2021,2(9):1441-1454. doi: 10.34067/KID.0003772021
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  • Ranea-Robles P, Chen H, Stauffer B, et al. The peroxisomal transporter ABCD3 plays a major role in hepatic dicarboxylic fatty acid metabolism and lipid homeostasis. J Inherit Metab Dis. 2021,44(6):1419-1433. doi: 10.1002/jimd.12440
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  • Ranea-Robles P, Violante S, Argmann C, et al. Murine deficiency of peroxisomal L-bifunctional protein (EHHADH) causes medium-chain 3-hydroxydicarboxylic aciduria and perturbs hepatic cholesterol homeostasis. Cell Mol Life Sci. 2021,78(14):5631-5646. doi: 10.1007/s00018-021-03869-9
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  • Violante S, Achetib N, van Roermund CWT, et al. Peroxisomes can oxidize medium- and long-chain fatty acids through a pathway involving ABCD3 and HSD17B4. FASEB J. 2019,33(3):4355-4364. doi: 10.1096/fj.201801498R
    Read this paper