NP_001189787.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
63,563 Da
NCBI Official Full Name
extracellular matrix protein 1 isoform 3
NCBI Official Synonym Full Names
extracellular matrix protein 1
NCBI Official Synonym Symbols
NCBI Protein Information
extracellular matrix protein 1; secretory component p85
UniProt Protein Name
Extracellular matrix protein 1
UniProt Synonym Protein Names
Secretory component p85
UniProt Entry Name
ECM1_HUMAN
NCBI Summary for ECM1
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]
UniProt Comments for ECM1
ECM1: Involved in endochondral bone formation as negative regulator of bone mineralization. Stimulates the proliferation of endothelial cells and promotes angiogenesis. Inhibits MMP9 proteolytic activity. Defects in ECM1 are the cause of lipoid proteinosis (LiP); 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. 4 isoforms of the human protein are produced by alternative splicing.
Protein type: Secreted, signal peptide; Secreted
Chromosomal Location of Human Ortholog: 1q21
Cellular Component: extracellular matrix; extracellular space; proteinaceous extracellular matrix
Molecular Function: protein C-terminus binding; signal transducer activity; protein binding; enzyme binding; protease binding; interleukin-2 receptor binding; laminin binding
Biological Process: ossification; positive regulation of I-kappaB kinase/NF-kappaB cascade; negative regulation of peptidase activity; signal transduction; regulation of transcription from RNA polymerase II promoter; negative regulation of bone mineralization; positive regulation of angiogenesis; biomineral formation; negative regulation of cytokine and chemokine mediated signaling pathway; positive regulation of endothelial cell proliferation; angiogenesis; regulation of T-helper 2 type immune response; inflammatory response
Disease: Lipoid Proteinosis Of Urbach And Wiethe
Research Articles on ECM1
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Diseases associated with ECM1 elisa kit
Organs/Tissues associated with ECM1 elisa kit
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