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Protein Page:
LAMB2 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitylation
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
LAMB2 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. Defects in LAMB2 are the cause of Pierson syndrome (PIERSS); also known as microcoria-congenital nephrotic syndrome. Pierson syndrome is characterized by nephrotic syndrome with neonatal onset, diffuse mesangial sclerosis and eye abnormalities with microcoria as the leading clinical feature. Death usually occurs within the first weeks of life. Disease severity depends on the mutation type: nontruncating LAMB2 mutations may display variable phenotypes ranging from a milder variant of Pierson syndrome to isolated congenital nephrotic syndrome. Defects in LAMB2 are the cause of nephrotic syndrome type 5 with or without ocular abnormalities (NPHS5). NPHS5 is a renal disease characterized clinically by proteinuria, hypoalbuminemia, hyperlipidemia and edema. Kidney biopsies show non-specific histologic changes such as focal segmental glomerulosclerosis and diffuse mesangial proliferation. Some affected individuals have an inherited steroid-resistant form and progress to end-stage renal failure. NPHS5 is characterized by very early onset of progressive renal failure. A subset of patients may develop mild ocular anomalies, such as myopia, nystagmus, and strabismus. Note: This description may include information from UniProtKB.
Protein type: Motility/polarity/chemotaxis; Extracellular matrix; Secreted, signal peptide; Secreted
Chromosomal Location of Human Ortholog: 3p21
Cellular Component: basal lamina; basement membrane; extracellular matrix; extracellular region; laminin-3 complex
Biological Process: extracellular matrix organization and biogenesis
Disease: Nephrotic Syndrome, Type 5, With Or Without Ocular Abnormalities; Pierson Syndrome
Reference #:  P55268 (UniProtKB)
Alt. Names/Synonyms: LAMB2; Laminin B1s chain; laminin S; Laminin subunit beta-2; laminin, beta 2 (laminin S); Laminin-11 subunit beta; Laminin-14 subunit beta; Laminin-15 subunit beta; Laminin-3 subunit beta; Laminin-4 subunit beta; Laminin-7 subunit beta; Laminin-9 subunit beta; LAMS; S-LAM beta; S-laminin subunit beta
Gene Symbols: LAMB2
Molecular weight: 195,981 Da
Basal Isoelectric point: 6.07  Predict pI for various phosphorylation states
Select Structure to View Below

LAMB2

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



 LTP 

LTP: 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.


 HTP 

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


       human

 
0 1 R170 ERSADFGRTWHVYRY
0 1 R176 GRTWHVYRYFSYDCG
0 1 Y232‑p AIPIPDPySSRIQNL
0 1 T477‑p CQCNARGtVPGSTPC
0 1 S614‑p TLEFLVAsVPKAMDY
0 1 E1271 ELRREIGEATEHLTQ
0 1 K1322 DQHLDLLKHSNFLGA
0 1 S1472‑p RALAEGGsILsRVAE
0 1 S1475‑p AEGGsILsRVAETRR
0 1 S1654‑p AGAERALssAGERAR
0 1 S1655‑p GAERALssAGERARQ
0 1 K1714‑ub GDQYQTVkALAERkA
0 1 K1720‑sm VkALAERkAQGVLAA
0 1 Y1759‑p LQELEGTyEENERAL
0 1 K1769‑ub NERALESkAAQLDGL
  mouse

 
R173 ERSADFGRTWHVYRY
R179 GRTWHVYRYFSYDCG
Y235 AIPIPDPYSSRIQNL
T480 CQCNSRGTVPGSSPC
S617 EVEFLVTSLPRAMDY
K1272 GLRHEIGKTTERLTQ
K1323 DQHLEILKHSNFLGA
G1473 RALVEGGGILSRVSE
S1476 VEGGGILSRVSETRR
N1655 AGAEKSLNSAGERAR
S1656 GAEKSLNSAGERARQ
R1715 GDQYQTVRALAERKA
K1721 VRALAERKAEGVLAA
Y1760 LQELEGTYEENERAL
K1770 NERALEGKAAQLDGL
  rat

 
R173‑m1 ERSADFGrTWRVYrY
R179‑m1 GrTWRVYrYFSYDCG
Y235 AIPIPDPYSSRIQNL
T480 CQCNSRGTVPGGTPC
S617 EVEFLVTSLPRAMDY
K1274‑ac GLRHEIGkTTERLTQ
K1325‑ac DQHLDILkHSNFLGA
G1475 RALVEGGGILSRVSE
S1478 VEGGGILSRVSETRR
N1657 AGTEQSLNSASERAR
S1658 GTEQSLNSASERARQ
R1717 GDQYQTVRALAERKA
K1723 VRALAERKAEGVLAA
Y1762 LQELEGTYEENEREL
K1772 NERELEVKAAQLDGL
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