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Protein Page:
COL4A3 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
COL4A3 Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. Autoantibodies against the NC1 domain of alpha 3(IV) are found in Goodpasture syndrome, an autoimmune disease of lung and kidney. Defects in COL4A3 are a cause of Alport syndrome autosomal recessive (APSAR). APSAR is characterized by progressive glomerulonephritis, glomerular basement membrane defects, renal failure, sensorineural deafness and specific eye abnormalities (lenticonous and macular flecks). The disorder shows considerable heterogeneity in that families differ in the age of end-stage renal disease and the occurrence of deafness. Defects in COL4A3 are a cause of benign familial hematuria (BFH); also known as thin basement membrane nephropathy. BFH is characterized by persistent hematuria, an electron microscopically detectable thin glomerular basement membrane (GBM) and an autosomal dominant mode of inheritance. Renal function remains normal. In children, differentiation between BFH and AS can be difficult, because both disorders are manifested by persistent hematuria and thin GBM at that age. Defects in COL4A3 are a cause of Alport syndrome autosomal dominant (APSAD). Alport syndrome is characterized by progressive glomerulonephritis, glomerular basement membrane defects, renal failure, sensorineural deafness and specific eye abnormalities (lenticonous and macular flecks). The disorder shows considerable heterogeneity in that families differ in the age of end-stage renal disease and the occurrence of deafness. Belongs to the type IV collagen family. 5 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Secreted, signal peptide; Secreted
Cellular Component: endoplasmic reticulum lumen; collagen type IV; extracellular region; basement membrane
Molecular Function: metalloendopeptidase inhibitor activity; integrin binding; protein binding; extracellular matrix structural constituent; structural molecule activity
Biological Process: caspase activation; axon guidance; extracellular matrix organization and biogenesis; blood circulation; glomerular basement membrane development; cell proliferation; extracellular matrix disassembly; collagen catabolic process; negative regulation of cell proliferation; negative regulation of angiogenesis; cell surface receptor linked signal transduction; sensory perception of sound; response to glucose stimulus; cell adhesion
Reference #:  Q01955 (UniProtKB)
Alt. Names/Synonyms: CO4A3; COL4A3; Collagen alpha-3(IV) chain; collagen IV, alpha-3 polypeptide; collagen, type IV, alpha 3 (Goodpasture antigen); Goodpasture antigen; Tumstatin
Gene Symbols: COL4A3
Molecular weight: 161,812 Da
Basal Isoelectric point: 9.28  Predict pI for various phosphorylation states
Select Structure to View Below

COL4A3

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 T235-p ERGVKGLtGPPGPPG
0 1 T243-p GPPGPPGtVIVTLtG
0 1 T249-p GtVIVTLtGPDNRTD
0 1 S382-p GPPGVPGsPGSSRPG
0 1 T835-p QQGRRGKtGPKGDPG
0 1 K924 SPGIPGVKGQRGTPG
0 1 K933 QRGTPGAKGEQGDKG
0 1 K1114 GSPGLPGKPGPHGDL
0 1 K1124 PHGDLGFKGIKGLLG
0 1 K1127 DLGFKGIKGLLGPPG
0 1 K1206-m2 FPGLPGRkGAMGDAG
0 1 S1246-p GNRGPPGsRGsPGAP
0 1 S1249-p GPPGsRGsPGAPGPP
1 1 K1414-ub EKGNKGSkGEPGPAG
2 0 S1435-p LKGKRGDsGSPATWT
0 1 T1443-p GSPATWTtRGFVFTR
1 0 S1452-p GFVFTRHsQTTAIPS
0 1 S1576 PCPHGWISLWKGFSF
0 1 S1648-p MFRKPIPstVKAGEL
0 1 T1649-p FRKPIPstVKAGELE
  mouse

 
T235 ERGMKGLTGPPGPPG
T243 GPPGPPGTVIFTLTQ
T249 GTVIFTLTQPYNKSD
S382 GPPGVPGSPGLSRPG
T833 QPGRRGDTGPKGDPG
K922-ac SLGIPGMkGEKGRPG
K931-ac EKGRPGAkGERGEKG
K1112-ac GSPGHPGkPGPAGDL
K1122-ac PAGDLGLkGQkGFPG
K1125-ac DLGLkGQkGFPGPPG
K1204 LSGLPGRKGVMGDVG
L1244 GDRGLPGLRGNPGEP
N1247 GLPGLRGNPGEPGPP
K1412 TKGNKGLKGQQGPPG
R1433 LKGNPGDRGTPATGT
M1442 TPATGTRMRGFIFTR
S1451 GFIFTRHSQTTAIPS
S1575-p PCPQDWVsLWKGFSF
S1647 MFRKPIPSTVKAGDL
T1648 FRKPIPSTVKAGDLE
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