• ECM1 Polyclonal Antibody
1 1

Upingbio

YP-Ab-03833-100UL

ECM1 Polyclonal Antibody

ECM1 Polyclonal Antibody

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  • Gene Name: ECM1
  • Immunogen: Synthesized peptide derived from the N-terminal region of human ECM1.
  • Specificity: ECM1 Polyclonal Antibody detects endogenous levels of ECM1 protein.
  • Composition: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • Source: Polyclonal, Rabbit,IgG
  • Dilution ratio: IHC: 1/100 - 1/300. ELISA: 1/20000.. IF 1:50-200
  • Purification process: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • Concentration: 1 mg/ml
  • Storage: -20°C/1 year
  • Other Names: ECM1; Extracellular matrix protein 1; Secretory component p85
  • Background: This gene encodes a soluble protein that is involved in endochondral bone formation, angiogenesis, and tumor biology. It also interacts with a variety of extracellular and structural proteins, contributing to the maintenance of skin integrity and homeostasis. Mutations in this gene are associated with lipoid proteinosis disorder (also known as hyalinosis cutis et mucosae or Urbach-Wiethe disease) that is characterized by generalized thickening of skin, mucosae and certain viscera. Alternatively spliced transcript variants encoding distinct isoforms have been described for this gene. [provided by RefSeq, Feb 2011],
  • Function: disease:Defects in ECM1 are the cause of lipoid proteinosis (LiP) [MIM:247100]; also known as lipoid proteinosis of Urbach and Wiethe or hyalinosis cutis et mucosae. LiP is a rare autosomal recessive disorder characterized by generalized thickening of skin, mucosae and certain viscera. Classical features include beaded eyelid papules and laryngeal infiltration leading to hoarseness. Histologically, there is widespread deposition of hyaline material and disruption/reduplication of basement membrane.,
  • Species: Human;Rat;Mouse;
  • Range: IHC;IF;ELISA
  • Protein: Extracellular matrix protein 1
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