SG196_HUMAN » Probable inactive protein kinase-like protein SgK196

SG196_HUMAN » Probable inactive protein kinase-like protein SgK196
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Topology in Endoplasmic reticulum membrane
Topologylumenal side
cytoplasmic side
SG196_HUMAN » Probable inactive protein kinase-like protein SgK196 » Sugen kinase 196;
Hydrophobic Thickness 34.0 ± 3.3 Å
Tilt Angle 30 ± 0°
ΔGtransfer -18.3 kcal/mol
ΔGfold -8.3 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC
Topology In
TM Segments 19-43 (19-44)
Pathways none
PDB none
OPM none
Complexes none
Interactions

BMR1B, Complex: SG196:BMR1B, PubMed

TGFR1, Complex: SG196:TGFR1, PubMed

Domains

AA: 81-219, PDBID: 3SXR, Subunit B, Seq Identity:37%, Protein tyrosine kinase

UniProt annotation for SG196_HUMAN » Probable inactive protein kinase-like protein SgK196
FUNCTION: Protein O-mannose kinase that specifically mediates phosphorylation at the 6-position of an O-mannose of the trisaccharide (N-acetylgalactosamine (GalNAc)-beta-1,3-N- acetylglucosamine (GlcNAc)-beta-1,4-mannose) to generate phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine- beta-1,3-N-acetylglucosamine-beta-1,4-(phosphate-6-)mannose). Phosphorylated O-mannosyl trisaccharide is a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity. Only shows kinase activity when the GalNAc-beta-3-GlcNAc-beta-terminus is linked to the 4-position of O-mannose, suggesting that this disaccharide serves as the substrate recognition motif.

CATALYTIC ACTIVITY: ATP + O(3)-(N-acetyl-beta-D-galactosaminyl- (1->3)-N-acetyl-beta-D-glucosaminyl-(1->4)-alpha-D-mannosyl)-L- threonyl/L-seryl-[protein] = ADP + O(3)-(N-acetyl-beta-D- galactosaminyl-(1->3)-N-acetyl-beta-D-glucosaminyl-(1->4)-alpha-D- (6-phospho)mannosyl)-L-threonyl/L-seryl-[protein].

TISSUE SPECIFICITY: Highest expression is observed in brain, skeletal muscle, kidney and heart in fetal and adult tissues.

DISEASE: Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A12 (MDDGA12) OMIM: An autosomal recessive disorder characterized by congenital muscular dystrophy associated with cobblestone lissencephaly and other brain anomalies, eye malformations, profound mental retardation, and death usually in the first years of life. Included diseases are the more severe Walker-Warburg syndrome and the slightly less severe muscle-eye-brain disease. Note=The disease is caused by mutations affecting the gene represented in this entry.

DISEASE: Muscular dystrophy-dystroglycanopathy limb-girdle C12 (MDDGC12) OMIM: An autosomal recessive limb-girdle congenital muscular dystrophy, characterized by muscle weakness and delayed motor development in association with cognitive impairment. caused by mutations affecting the gene represented in this entry.

UniProt features for SG196_HUMAN » Probable inactive protein kinase-like protein SgK196
CHAIN 1 350 Probable inactive protein kinase-like protein SgK196.
DOMAIN 81 350 Protein kinase.
Amino Acid Sequence for SG196_HUMAN » Probable inactive protein kinase-like protein SgK196
MEKQPQNSRR GLAPREVPPA VGLLLIMALM NTLLYLCLDH FFIAPRQSTV DPTHCPYGHF RIGQMKNCSP WLSCEELRTE VRQLKRVGEG AVKRVFLSEW KEHKVALSQL TSLEMKDDFL HGLQMLKSLQ GTHVVTLLGY CEDDNTMLTE YHPLGSLSNL EETLNLSKYQ NVNTWQHRLE LAMDYVSIIN YLHHSPVGTR VMCDSNDLPK TLSQYLLTSN FSILANDLDA LPLVNHSSGM LVKCGHRELH GDFVAPEQLW PYGEDVPFHD DLMPSYDEKI DIWKIPDISS FLLGHIEGSD MVRFHLFDIH KACKSQTPSE RPTAQDVLET YQKVLDTLRD AMMSQAREML