Product Name	FACE1/ZMPSTE24
Chinese Name	锌金属蛋白酶STE24同源蛋白抗体
Alias	CAAX prenyl protease 1 homolog; FACE-1; FACE1; FACE1_HUMAN; Farnesylated proteins converting enzyme 1; Farnesylated proteins-converting enzyme 1; Prenyl protein specific endoprotease 1; Prenyl protein-specific endoprotease 1; STE24; Zinc metalloproteinase Ste24 homolog; zmpste24.
Research Area	Cell biology  Developmental biology  transcriptional regulatory factor  Zinc finger protein  Epigenetics
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep, )
Applications	ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	55kDa
Cellular localization	The nucleus cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human FACE1/ZMPSTE24: 381-475/475
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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] Function: Proteolytically removes the C-terminal three residues of farnesylated proteins. Acts on lamin A/C. Subcellular Location: Endoplasmic reticulum membrane. Golgi apparatus membrane. Tissue Specificity: Widely expressed. High levels in kidney, prostate, testis and ovary. 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. Similarity: Belongs to the peptidase M48A family. SWISS: O75844 Gene ID: 10269 Database links: Entrez Gene: 10269 Human Omim: 606480 Human SwissProt: O75844 Human Unigene: 132642 Human Unigene: 721062 Human

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