ZMPSTE24, Polyclonal Antibody

CAAX prenyl protease 1 homolog; FACE-1; Farnesylated proteins-converting enzyme 1; Prenyl protein specific endoprotease 1; STE24; Zinc metalloproteinase Ste24 homolog; zmpste24 antibody

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

Polyclonal

Rabbit

Affinity Purified

0.55 ug/ul in antibody stabilization buffer (lot specific)

-20 degree C for long term storage

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.

Affinity Purified CAAX prenyl protease 1 homolog Antibody
Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C.

Primary Antibodies; Signal Transduction; Cytoskeleton / ECM; Intermediate Filaments; ZMPSTE24 Antibody

ELISA (EIA), Western Blot (WB)

Dot Blot: 1:10,000
ELISA: 1:10,000
Western Blot: 1:500

54,735 Da

zinc metallopeptidase, STE24

CAAX prenyl protease 1 homolog

CAAX prenyl protease 1 homolog

Face1; FACE-1  [Similar Products]

FACE1_MOUSE

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

Cellular Component: endoplasmic reticulum; integral to membrane; membrane; nucleus

Molecular Function: hydrolase activity; metal ion binding; metalloendopeptidase activity; metallopeptidase activity; peptidase activity

Biological Process: nuclear membrane organization and biogenesis; prenylated protein catabolic process; proteolysis

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