Rabbit Extracellular Matrix Protein 1 ELISA Kit | ECM1 elisa kit
Rabbit Extracellular Matrix Protein 1 ELISA Kit
NCBI and Uniprot Product Information
Uniprot Description
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; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 1q21
Cellular Component: extracellular matrix; extracellular space; proteinaceous extracellular matrix
Molecular Function: protein C-terminus binding; protein binding; signal transducer activity; 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; positive regulation of endothelial cell proliferation; negative regulation of cytokine and chemokine mediated signaling pathway; angiogenesis; inflammatory response; regulation of T-helper 2 type immune response
Disease: Lipoid Proteinosis Of Urbach And Wiethe