Goat anti-FACE1/ZMPSTE24 Antibody | MyBioSource MBS420669 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Goat anti-FACE1/ZMPSTE24 Antibody

catalog :

MBS420669

quantity :

0.1 mg

price :

300 USD

clonality :

polyclonal

host :

goat

conjugate :

nonconjugated

reactivity :

human

application :

western blot, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS420669

products type :

Antibody

products full name :

Goat anti-FACE1/ZMPSTE24 Antibody

products short name :

FACE1/ZMPSTE24

products name syn :

FACE1; ZMPSTE24; zinc metallopeptidase (STE24 homolog, yeast); FLJ14968; MADB; STE24; Ste24p; CAAX prenyl protease; farnesylated-proteins converting enzyme 1; prenyl protein-specific endoprotease 1; zinc metalloproteinase (STE24 homolog, yeast); zinc met; FACE1 antibody; ZMPSTE24 antibody; zinc metallopeptidase (STE24 homolog; yeast) antibody; FLJ14968 antibody; MADB antibody; STE24 antibody; Ste24p antibody; CAAX prenyl protease antibody; farnesylated-proteins converting enzyme 1 antibody; prenyl protein-specific endoprotease 1 antibody; zinc metalloproteinase (STE24 homolog; yeast) antibody; zinc metalloproteinase STE24 homolog antibody; zinc metalloproteinase; STE24 (yeast; homolog) antibody; FACE-1 antibody; FACE1 / ZMPSTE24

other names :

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

products gene name :

ZMPSTE24

products gene name syn :

FACE1

other gene names :

ZMPSTE24; ZMPSTE24; HGPS; PRO1; FACE1; STE24; FACE-1; Ste24p; FACE1; STE24; FACE-1

uniprot entry name :

FACE1_HUMAN

clonality :

Polyclonal

host :

Goat

reactivity :

Tested: Human; Expected from sequence similarity: Human

sequence length :

475

sequence :

ERLQALKTMKQH

purity :

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide

form :

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

concentration :

100ug specific antibody in 200ul

storage stability :

Aliquot and store at -20 degree C. Minimize freezing and thawing.

tested application :

Peptide ELISA (EIA), Western Blot (WB)

app notes :

Peptide ELISA: Antibody detection limit dilution 1: 128000. Western Blot: Approx 55kDa band observed in Human Skin lysates (calculated MW of 54.8kDa according to NP_005848.2). Recommended concentration: 0.5-1.5ug/ml.

other info1 :

Immunogen: Peptide with sequence C-ERLQALKTMKQH, from the C Terminus of the protein sequence according to NP_005848.2. Epitope: C Terminus

ncbi gi num :

18379366

ncbi acc num :

NP_005848.2

ncbi gb acc num :

NM_005857.4

ncbi mol weight :

54,813 Da

ncbi pathways :

Adipogenesis Pathway (198832); Terpenoid Backbone Biosynthesis Pathway (83022); Terpenoid Backbone Biosynthesis Pathway (408)

ncbi summary :

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]

uniprot summary :

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: Protease; Membrane protein, multi-pass; EC 3.4.24.84; Membrane protein, integral. Chromosomal Location of Human Ortholog: 1p34. Cellular Component: endoplasmic reticulum membrane; integral to membrane; membrane; nuclear inner membrane. Molecular Function: metal ion binding; metalloendopeptidase activity; metalloexopeptidase activity. Biological Process: nuclear membrane organization and biogenesis; prenylated protein catabolic process; proteolysis. Disease: Mandibuloacral Dysplasia With Type B Lipodystrophy; Restrictive Dermopathy, Lethal

size1 :

0.1 mg

price1 :

300 USD

more info or order :

MyBioSource product webpage