Overview

  • Product name

  • Description

    Rabbit polyclonal to ZMPSTE24
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-P, ELISAmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Synthetic peptide (10-30 amino acids in length) conjugated to KLH, from the region of amino acids 415-430 of Human ZMPSTE24.

  • Positive control

    • Human breast carcinoma tissue. WB: A431 cell lysate and human kidney tissue lysate.

Properties

Applications

Our Abpromise guarantee covers the use of ab38450 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 1/100 - 1/500. Detects a band of approximately 55 kDa (predicted molecular weight: 55 kDa).
IHC-P 1/50 - 1/100.
ELISA 1/1000.

Target

  • Function

    Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C.
  • Tissue specificity

    Widely expressed. High levels in kidney, prostate, testis and ovary.
  • Involvement in disease

    Defects in ZMPSTE24 are the cause of mandibuloacral dysplasia with type B lipodystrophy (MADB) [MIM:608612]. Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and types A or B patterns of lipodystrophy. Type B lipodystrophy observed in MADB, is characterized by generalized fat loss.
    Defects in ZMPSTE24 are a cause of lethal tight skin contracture syndrome (LTSCS) [MIM:275210]; also called restrictive dermopathy (RD). Lethal tight skin contracture syndrome is a rare disorder mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial features (small mouth, small pinched nose and micrognathia), sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life. The overall prevalence of consanguineous cases suggested an autosomal recessive inheritance.
  • Sequence similarities

    Belongs to the peptidase M48A family.
  • Cellular localization

    Endoplasmic reticulum membrane. Golgi apparatus membrane.
  • Information by UniProt
  • Database links

  • Alternative names

    • CAAX prenyl protease 1 homolog antibody
    • FACE-1 antibody
    • FACE1 antibody
    • FACE1_HUMAN antibody
    • Farnesylated proteins converting enzyme 1 antibody
    • Farnesylated proteins-converting enzyme 1 antibody
    • Prenyl protein specific endoprotease 1 antibody
    • Prenyl protein-specific endoprotease 1 antibody
    • STE24 antibody
    • Zinc metalloproteinase Ste24 homolog antibody
    • zmpste24 antibody
    see all

Images

  • All lanes : Anti-ZMPSTE24 antibody (ab38450) at 1/1000 dilution

    Lane 1 : A431 whole cell lysate
    Lane 2 : Human kidney tissue lysate

    Lysates/proteins at 20 µg per lane.

    Secondary
    All lanes : Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution

    Predicted band size: 55 kDa

  • ab38450, at 1/50 dilution, staining ZMPSTE24 in Human breast carcinoma by Immunohistochemistry, Formalin fixed, Paraffin embedded tissue. ab38450 was peroxidase-conjugated to the secondary antibody, followed by AEC staining.

References

ab38450 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

Answer

Thank you for contacting us.

ab38450 recognises aa415-430 of human ZMPSTE24.

I hope this information is helpful to you. Please do not hesitate to contact us if you need any more advice or information.

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