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Product Name FRAS1 Chinese Name Extracellular matrix蛋白FRAS1抗体 Alias Extracellular matrix protein FRAS1; Fras 1; Fras1; FRAS1_HUMAN; Fraser syndrome 1. Research Area Cardiovascular Cell biology Signal transduction Extracellular matrix Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, ) 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 440kDa Cellular localization The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human FRAS1: 1101-1200/4008 <Extracellular> Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. 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 an extracellular matrix protein that appears to function in the regulation of epidermal-basement membrane adhesion and organogenesis during development. Mutations in this gene cause Fraser syndrome, a multisystem malformation that can include craniofacial, urogenital and respiratory system abnormalities. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Oct 2009].
Subcellular Location:
Cell membrane; Single-pass type I membrane protein; Extracellular side (Potential).
Tissue Specificity:
Expressed in many adult tissues, with highest levels in kidney, pancreas and thalamus. Relatively high expression was also detected in fetal kidney and heart.
DISEASE:
Defects in FRAS1 are a cause of Fraser syndrome (FRASS) [MIM:219000]. Fraser syndrome is a multisystem malformation usually comprising cryptophthalmos, cutaneous syndactyly, ear abnormalities, renal agenesis and congenital heart defects.
Similarity:
Belongs to the FRAS1 family.
Contains 5 Calx-beta domains.
Contains 12 CSPG (NG2) repeats.
Contains 14 FU (furin-like) repeats.
Contains 6 VWFC domains.
SWISS:
Q86XX4
Gene ID:
80144
Database links:Entrez Gene: 80144 Human
Omim: 607830 Human
SwissProt: Q86XX4 Human
Unigene: 369448 Human
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