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FLASH GENE

Symbol

BGN

contributors: npt/mct/pgu - updated : 04-07-2016

HGNC name	biglycan
HGNC id	1044

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

DNA

TYPE	functioning gene
STRUCTURE	14.59 kb     8 Exon(s)

10 Kb 5' upstream gene genomic sequence study

regulatory sequence	Binding site   transcription factor
text structure	SP-1, AP-1, AP-2
	structure : no TATA, no CAAT
	housekeeping

MAPPING

cloned

Y

linked

status

confirmed

Map	cen - DXS304 - DXS305 ,DXS374 - GABRA3 - DXS52 - DXS15 - BGN - L1CAM - AVPR2 - ARD1A - DXS80 - RGCP@ - qter
Text	see F8 ,GABRA3

RNA

TRANSCRIPTS	type	messenger

text	multiple isoforms in lung fibroblasts differing in polysaccharide substitution

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_001711 8 - 2401 NP_001702 - 368 - 2002 12153565 variant 1

EXPRESSION

Type	ubiquitous

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol blood / hematopoietic spleen         Digestive liver         Respiratory lung         Skeleton axial skeleton       highly Homo sapiens

tissue

System Tissue Tissue level 1 Tissue level 2 Level Pubmed Species Stage Rna symbol Connective bone     highly Homo sapiens

cells

System Cell Pubmed Species Stage Rna symbol   digestive not specific chondroblast Homo sapiens Skeleton osteoblast Homo sapiens Skin/Tegument keratinocyte

cell lineage

cell lines

fluid/secretion

at STAGE

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	a N terminal O-linked glycosylation site
	ten leucine-rich repeats flanked by cysteine-rich regions
	a N-linked glycosylation site (NKSIT motifs) in the LRR region

HOMOLOGY

Homologene

FAMILY	small leucine-rich proteoglycan (SLRP) family
	class I subfamily

CATEGORY

structural protein

SUBCELLULAR LOCALIZATION	extracellular
	intracellular
	intracellular,cytoplasm,organelle,lumen
	intracellular,cytoplasm,organelle,Golgi
	intracellular,cytoplasm,organelle,lysosome
text	extracellular matrix

basic FUNCTION	may be function))ing in connective tissue metabolism by binding to collagen fibrils and transfering growth factor-beta
	be may promoting neuronal survival
	with decorin have a coordinate and cooperative relationship in the regulation of stromal fibrillogenesis
	plays an important role in the highly orchestrated process of dentin mineralization
	WISP1 and BGN may functionally interact and control each other activity, thus regulating the differentiation and proliferation of osteogenic cells
	required for stable collagen matrix formation of infarct scars and for preservation of cardiac hemodynamic function
	promotes osteoblast differentiation and this function is due in part to its ability to positively modulate bone morphogenetic protein (BMP) functions
	could facilitate osteoblast functions
	BGN core protein is responsible for its function in promoting the BMP2 functions and the glycosaminoglycan component could function as a suppressor in this process
	promote bone morphogenetic protein-4 (BMP4) stimulated osteoblastic differentiation
	BGN and DCN may have sequential roles in the tendon response to injury
	BGN signaling supported fetal membrane remodeling during early gestation in the absence of concomitant changes in TGFB1 levels
	BGN has likely a regulatory role in the process of bone formation during fracture healing, and further, that reduced angiogenesis could be the molecular basis
	is a factor secreted by peritubular cells to modulate FGFR3c signaling and thus contributes to the regulation of spermatogonial maintenance
	BGN and DCN are class I members of the small leucine-rich proteoglycan (SLRP) family that regulate collagen fibril size
	BGN is a CADASIL-enriched protein that potentially accumulates in vessels by MTOR-mediated transcriptional activation and/or post-translational accumulation via protein interactions with NOTCH3 and collagen
	extracellular matrix proteoglycan that can bind transforming growth factor beta (TGFB1)
	is an important structural component of articular cartilage and participates in the assembly of the chondrocyte extracellular matrix through the formation of protein interactions with type VI collagen and large proteoglycan aggregates

CELLULAR PROCESS

PHYSIOLOGICAL PROCESS

PATHWAY

metabolism

signaling

a component

INTERACTION

DNA

RNA

small molecule

cofactor,

protein	binding collagen fibrils and transferring TGFB1
	interaction with DCN during corneal development and decorin may have a primary role in regulating fibril assembly, a function that can be fine-tuned by biglycan during early development
	FGFR3 facilitates BMPR1a to degradation through Smurf1-mediated ubiquitination pathway
	BGN binds to FGFR3c and FGF1

cell & other

REGULATION

Other

regulated by matriuretic peptide precursor C for the mineralization of loss

ASSOCIATED DISORDERS

corresponding disease(s)

SEMD6

Other morbid association(s)

Type Gene Modification Chromosome rearrangement Protein expression Protein Function constitutional       loss of function increases aortic aneurysm development and is not protective against the development of atherosclerosis, leading to loosely packed aortic collagen fibers

Susceptibility

to carpal tunnel syndrome (CTS)

Variant & Polymorphism other	variants within the BGN gene, independently and by interacting with COL5A1 variants, are associated with CTS

Candidate gene
Marker
Therapy target
	System Type Disorder Pubmed reproduction pregnancy   novel target for research on preventive strategies for preterm premature rupture of fetal membranes

ANIMAL & CELL MODELS

	targeted disruption of Bgn in mice,lead to an osteoporotic like phenotype
	biglycan-deficient mice exhibited significantly increased vascular perlecan content compared to biglycan wildtype mice