Anti-ZMPSTE24 Antibody (FITC)
Rabbit polyclonal antibody to ZMPSTE24 (FITC) from FabGennix (ZMPSTE24.112-FITC).
|Name:||Anti-ZMPSTE24 Antibody (FITC)|
|Description:||Rabbit polyclonal antibody to ZMPSTE24 (FITC)|
|Dilutions:||DB: 1:10,000; ELISA: 1:10,000; Western Blot: 1:500|
|Immunogen:||Synthetic peptide taken within amino acid region 450-480 on mouse ZMPSTE24 protein.|
|Concentration:||0.55 µg/µl in antibody stabilization buffer|
|Storage:||-20⁰C for long term storage|
|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:||Mandibuloacral dysplasia with type B lipodystrophy: A disorder characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and generalized lipodystrophy with loss of subcutaneous fat from the extremities, face, neck and trunk.
Lethal tight skin contracture syndrome: 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 Location:||Endoplasmic reticulum membrane. Nucleus inner membrane.|
|Synonyms:||CAAX prenyl protease 1 homolog 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
Zinc metalloproteinase Ste24 homolog Antibody
|Information:||Target information shown above is from the UniProt Consortium.|
Please Note: Anti-ZMPSTE24 Antibody (FITC) is for research use only. It is not intended for diagnostic of therapeutic use.