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. Author manuscript; available in PMC: 2009 Jan 19.
Published in final edited form as: Front Biosci. 2008 May 1;13:6455–6471. doi: 10.2741/3166

Table 3.

Motifs for posttranslational modifications mutated in disease

Phosphorylation sites
Liver Glycogenosis Type II Phosphorylase kinase RxxT→ RxxI Phosphorylation site LOF (76)
Pleiotropic Malformation Syndrome STRA6 RxT→ CxT PKA phosphorylation site LOF (48)
Maturity-onset Diabetes of the Young HNF4α RxG→RxS PKA phosphorylation site GOF (73)
Schizophrenia Synapsin III SP→NP MAPK phosphorylation site LOF (75)
Bukitt’s Lymphoma cMyc PTxxxS→ several GSK3 phosphoylation site LOF (74)
Familial Advanced Sleep Phase Syndrome Per2 S662G within binding site CKIε binding site LOF (72)
Familial Dysautonomia IKAP RIVT→PIVT CamKII phosphorylation Site LOF (77)
Protease Cleavage sites
Rickets FGF-23 RxxRQxxR, RxxQ, RxxW Protease cleavage site LOF (79)
Hypohidrotic Ectodermal Dysplasia ectodysplasin-A RxKR→ RxNR, CxKR, HxKR Furin cleavage site LOF (81)
Intrauterine Growth Retardation IGF1 Receptor RKRR→RKQR Protease cleavage site LOF (80)
Nonsyndromic Deafness Low density-lipoprotein receptor A RIVGG→LIVGG Protease cleavage site LOF (86)
Haemophilia Factor VIII R372C, R1689C Protein C, Thrombin cleavage site LOF (84, 85)
N-linked glycosylation
(Many diseases) ----- ----- N-glycosylation site GOF (89)
Retinitis Pigmentosa Rhodopsin NxS→NxN N-glycosylation site LOF (90)
Metachromatic Leukodystrophy Sphingolipid Activator protein B NxT→HxT N-glycosylation site LOF (92)
Creutzfeld-Jacob disease Prion NxT→NxA N-glycosylation site LOF (91)