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Rabbit Anti-Laminin subunit beta-4/LAMB4/FITC Conjugated antibody
background:
Binding to cells via a high affinity receptor, laminin is thought to mediate the attachment, migration and organization of cells into tissues during embryonic development by interacting with other extracellular matrix components.
Function:
Receptor for the SLCtype natriuretic peptide NPPC/CNP hormone. Has guanylate cyclase activity upon binding of its ligand. May play a role in the regulation of skeletal growth.
Subcellular Location:
Secreted, extracellular space, extracellular matrix, basement membrane.
Post-translational modifications:
Phosphorylation of the protein kinase-like domain is required for full activation by CNP.
DISEASE:
Acromesomelic dysplasia, Maroteaux type (AMDM) [MIM:602875]: An autosomal recessive acromesomelic chondrodysplasia. Acromesomelic chondrodysplasias are rare hereditary skeletal disorders characterized by short stature, very short limbsand hand/foot malformations. The severity of limb abnormalities increases from proximal to distal with profoundly affected hands and feet showing brachydactyly and/or rudimentary fingers (knob-like fingers). AMDM is characterized by axial skeletal involvement with wedging of vertebral bodies. In AMDM all skeletal elements are present but show abnormal rates of linear growth. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the adenylyl cyclase class-4/guanylyl cyclase family.
Contains 1 guanylate cyclase domain.
Contains 1 protein kinase domain.
Database links:
Entrez Gene: 22798 Human
SwissProt: A4D0S4 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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