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Protein Page:
ACAN (human)

ACAN This proteoglycan is a major component of extracellular matrix of cartilagenous tissues. A major function of this protein is to resist compression in cartilage. It binds avidly to hyaluronic acid via an N-terminal globular region. Defects in ACAN are the cause of spondyloepiphyseal dysplasia type Kimberley (SEDK). Spondyloepiphyseal dysplasias are a heterogeneous group of congenital chondrodysplasias that specifically affect epiphyses and vertebrae. The autosomal dominant SEDK is associated with premature degenerative arthropathy. Defects in ACAN are the cause of spondyloepimetaphyseal dysplasia aggrecan type (SEMD-ACAN). A bone disease characterized by severe short stature, macrocephaly, severe midface hypoplasia, short neck, barrel chest and brachydactyly. The radiological findings comprise long bones with generalized irregular epiphyses with widened metaphyses, especially at the knees, platyspondyly, and multiple cervical-vertebral clefts. Defects in ACAN are the cause of osteochondritis dissecans short stature and early-onset osteoarthritis (OD). It is a type of osteochondritis defined as a separation of cartilage and subchondral bone from the surrounding tissue, primarily affecting the knee, ankle and elbow joints. It is clinically characterized by multiple osteochondritic lesions in knees and/or hips and/or elbows, disproportionate short stature and early-onset osteoarthritis. Belongs to the aggrecan/versican proteoglycan family. 3 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Cell adhesion; Extracellular matrix; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 15q26.1
Cellular Component: extracellular matrix; extracellular region; Golgi lumen; lysosomal lumen; proteinaceous extracellular matrix
Molecular Function: extracellular matrix structural constituent; hyaluronic acid binding; protein binding
Biological Process: cell adhesion; central nervous system development; extracellular matrix disassembly; extracellular matrix organization and biogenesis; keratan sulfate biosynthetic process; keratan sulfate catabolic process; skeletal development
Disease: Osteochondritis Dissecans, Short Stature, And Early-onset Osteoarthritis; Spondyloepimetaphyseal Dysplasia, Aggrecan Type; Spondyloepiphyseal Dysplasia, Kimberley Type
Reference #:  P16112 (UniProtKB)
Alt. Names/Synonyms: ACAN; AGC1; AGCAN; aggrecan; Aggrecan core protein; Aggrecan core protein 2; Cartilage-specific proteoglycan core protein; Chondroitin sulfate proteoglycan 1; Chondroitin sulfate proteoglycan core protein 1; CSPCP; CSPG1; CSPGCP; large aggregating proteoglycan; MSK16; PGCA; SEDK
Gene Symbols: ACAN
Molecular weight: 250,193 Da
Basal Isoelectric point: 4.1  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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