Basement membrane-specific heparan sulfate proteoglycan core protein
GeneName
HSPG2
Summary
HSPG2, also known as perlecan, is a large heparan sulfate proteoglycan with a mass of 469 kDa, primarily localised in the basement membrane and extracellular matrix. It plays a crucial role in maintaining the structural integrity of tissues and is involved in various biological processes such as angiogenesis, brain development, and cell differentiation. HSPG2 interacts with various proteins, including collagen and low-density lipoprotein particles, and is implicated in receptor-mediated endocytosis. Its presence in the extracellular space and association with focal adhesions highlight its importance in cell-matrix interactions and signalling pathways.
Importance
HSPG2 is relevant to: - Tissue repair and regeneration due to its role in organ regeneration and angiogenesis - The regulation of inflammatory responses, which can influence various pathological conditions - Brain development and function, making it a potential target for neurodevelopmental research - Vascular health through its involvement in endothelial cell proliferation and circulatory system development - The modulation of lipid metabolism, which has implications in metabolic disorders
Top Products
For researchers investigating HSPG2, we recommend two primary antibodies that cater to different experimental needs. The first is the well-cited monoclonal antibody, Anti-Heparan Sulfate Proteoglycan 2/Perlecan antibody [A7L6] (ab2501), which has garnered 39 citations and is particularly effective for immunohistochemistry (IHC). This product is highly regarded for its reliability in detecting HSPG2 in tissue samples. Additionally, we offer the recombinant antibody, Anti-Heparan Sulfate Proteoglycan 2/Perlecan antibody [EPR22844-82] (ab255829). This antibody is validated for use in Western blotting (WB) and provides the batch-to-batch consistency that researchers often seek in recombinant products. With its proven performance, this antibody is an excellent choice for those requiring robust detection of HSPG2 in their experiments. The Recombinant Human Heparan Sulfate Proteoglycan 2/Perlecan protein ELISA Kit (ab114285) is an excellent option for researchers looking to study HSPG2 in their experiments.
Abcam Product Citation Summary
The use of Abcam antibody ab2501 for detecting HSPG2 in ICC-IF highlights its application in studying decellularized kidney matrices in mouse models. This suggests a focus on tissue engineering and regenerative medicine.
Abcam Product Citation Table
Function
Integral component of basement membranes. Component of the glomerular basement membrane (GBM), responsible for the fixed negative electrostatic membrane charge, and which provides a barrier which is both size- and charge-selective. It serves as an attachment substrate for cells. Plays essential roles in vascularization. Critical for normal heart development and for regulating the vascular response to injury. Also required for avascular cartilage development (PubMed:12435733, PubMed:15591058, PubMed:19789387). In muscle, it is essential for localizing acetylcholinesterase (AChE) at the neuromuscular junctions (NMJ), most probably acting as an adapter that links the acetylcholinesterase collagenic tail peptide (COLQ) to alpha-dystroglycan, and is therefore involved in the down-regulation of colinergic synaptic transmission (By similarity).
Endorepellin
Anti-angiogenic and anti-tumor peptide that inhibits endothelial cell migration, collagen-induced endothelial tube morphogenesis and blood vessel growth in the chorioallantoic membrane. Blocks endothelial cell adhesion to fibronectin and type I collagen. Anti-tumor agent in neovascularization. Interaction with its ligand, integrin alpha2/beta1, is required for the anti-angiogenic properties. Evokes a reduction in phosphorylation of receptor tyrosine kinases via alpha2/beta1 integrin-mediated activation of the tyrosine phosphatase, PTPN6.
LG3 peptide
Has anti-angiogenic properties that require binding of calcium ions for full activity.
Involvement in disease
Schwartz-Jampel syndrome
SJS1
Rare autosomal recessive disorder characterized by permanent myotonia (prolonged failure of muscle relaxation) and skeletal dysplasia, resulting in reduced stature, kyphoscoliosis, bowing of the diaphyses and irregular epiphyses.
None
The disease is caused by variants affecting the gene represented in this entry.
Dyssegmental dysplasia Silverman-Handmaker type
DDSH
The dyssegmental dysplasias are rare, autosomal recessive skeletal dysplasias with anisospondyly and micromelia. There are two recognized types: the severe, lethal DDSH and the milder Rolland-Desbuquois form. Individuals with DDSH also have a flat face, micrognathia, cleft palate and reduced joint mobility, and frequently have an encephalocoele. The endochondral growth plate is short, the calcospherites (which are spherical calcium-phosphorus crystals produced by hypertrophic chondrocytes) are unfused, and there is mucoid degeneration of the resting cartilage.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
Proteolytic processing produces the C-terminal angiogenic peptide, endorepellin. This peptide can be further processed to produce the LG3 peptide.
O-glycosylated with core 1 or possibly core 8 glycans. Contains three heparan sulfate chains. Also contains chondroitin sulfate.
Tissue Specificity
Detected in cerebrospinal fluid, fibroblasts and urine (at protein level).
Cellular localization
- Secreted
- Extracellular space
- Extracellular matrix
- Basement membrane
- Secreted
Alternative names
Basement membrane-specific heparan sulfate proteoglycan core protein, HSPG, Perlecan, PLC, HSPG2