Rabbit Anti-FACE1/ZMPSTE24 antibody

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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 metallop

Cat:

SL4392R

Species Reactivity：

(predicted: Human,Mouse,Rat,Chicken,Dog,Cow,Horse,Rabbit,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human FACE1/ZMPSTE24:381-475/475

Format：

Liquid

Storage instructions：

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500ICC=1:100-500IF=1:100-500（Paraffin sections need to do antigen repair）not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

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Unit:

Price: $228.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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 SLCterminal 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: 60696 Human

SwissProt: O75844 Human

Unigene: 132642 Human

Unigene: 721062 Human

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