ZMPSTE24, Polyclonal Antibody

CAAX prenyl protease 1 homolog; Farnesylated proteins-converting enzyme 1; FACE-1; Prenyl protein-specific endoprotease 1; Zinc metalloproteinase Ste2; ZMPSTE24; FACE1; STE24

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

>95%, Protein G purified

Liquid

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-ZMPSTE24 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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

ELISA (EIA), Immunohistochemistry (IHC), Immunofluorescence (IF)

IHC: 1: 20-1: 200
IF: 1: 50-1: 200

Immunohistochemistry of paraffin-embedded human liver tissue using MBS7049404 at dilution of 1:100

Immunohistochemistry of paraffin-embedded human gastric cancer using MBS7049404 at dilution of 1:100

Immunofluorescent analysis of PC3 cells using MBS7049404 at a dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)

54,813 Da

zinc metallopeptidase STE24

CAAX prenyl protease 1 homolog

CAAX prenyl protease 1 homolog

FACE1; STE24; FACE-1  [Similar Products]

FACE1_HUMAN

This gene encodes a member of the peptidase M48A family. The encoded protein is a zinc metalloproteinase involved in the two step post-translational proteolytic cleavage of carboxy terminal residues of farnesylated prelamin A to form mature lamin A. Mutations in this gene have been associated with mandibuloacral dysplasia and restrictive dermopathy. [provided by RefSeq, Jul 2008]

ZMPSTE24: Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C. Defects in ZMPSTE24 are the cause of mandibuloacral dysplasia with type B lipodystrophy (MADB). 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); 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. Belongs to the peptidase M48A family.

Protein type: EC 3.4.24.84; Membrane protein, integral; Membrane protein, multi-pass; Protease

Chromosomal Location of Human Ortholog: 1p34

Cellular Component: integral to endoplasmic reticulum membrane; membrane

Molecular Function: metalloendopeptidase activity; metalloexopeptidase activity

Biological Process: proteolysis

Disease: Mandibuloacral Dysplasia With Type B Lipodystrophy; Restrictive Dermopathy, Lethal

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