Overview

  • Product name

  • Description

    Goat polyclonal to HMBS/PBGD
  • Host species

    Goat
  • Specificity

    ab210677 is expected to recognize all reported isoforms (NP_000181.2; NP_001019553.1; NP_001245137.1; NP_001245138.1).
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat, Cow
  • Immunogen

    Synthetic peptide corresponding to Human HMBS/PBGD aa 160-173 (internal sequence) (Cysteine residue). NP_000181.2.
    Sequence:

    HLEFRSIRGNLNTR


    Database link: P08397

  • Positive control

    • K562 cell lysate.
  • General notes

     This product was previously labelled as HMBS

     

Properties

Applications

Our Abpromise guarantee covers the use of ab210677 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB Use a concentration of 0.1 - 0.3 mg/ml. Predicted molecular weight: 39 kDa.

Target

  • Function

    Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
  • Tissue specificity

    Isoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.
  • Pathway

    Porphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 2/4.
  • Involvement in disease

    Defects in HMBS are the cause of acute intermittent porphyria (AIP) [MIM:176000]. AIP is a form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors.
  • Sequence similarities

    Belongs to the HMBS family.
  • Cellular localization

    Cytoplasm.
  • Information by UniProt
  • Database links

  • Alternative names

    • HEM3_HUMAN antibody
    • HMBS antibody
    • Hydroxymethylbilane synthase antibody
    • PBG D antibody
    • PBG-D antibody
    • PBGD antibody
    • PORC antibody
    • Porphobilinogen deaminase antibody
    • porphyria, acute; Chester type antibody
    • Pre uroporphyrinogen synthase antibody
    • Pre-uroporphyrinogen synthase antibody
    • UPS antibody
    • Uroporphyrinogen I synthase antibody
    • Uroporphyrinogen I synthetase antibody
    see all

Images

  • Anti-HMBS/PBGD antibody (ab210677) at 0.1 µg/ml + K562 cell lysate at 35 µg

    Developed using the ECL technique.

    Predicted band size: 39 kDa

References

ab210677 has not yet been referenced specifically in any publications.

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