HUTCHINSON GILFORD SYNDROME
\hˈʌt͡ʃɪnsən ɡˈɪlfəd sˈɪndɹə͡ʊm], \hˈʌtʃɪnsən ɡˈɪlfəd sˈɪndɹəʊm], \h_ˈʌ_tʃ_ɪ_n_s_ə_n ɡ_ˈɪ_l_f_ə_d s_ˈɪ_n_d_ɹ_əʊ_m]\
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An abnormal congenital condition characterized by premature aging in children, where all the changes of cell senescence occur. It is manifested by premature greying; hair loss; hearing loss (DEAFNESS); cataracts (CATARACT); ARTHRITIS; OSTEOPOROSIS; DIABETES MELLITUS; atrophy of subcutaneous fat; skeletal hypoplasia; and accelerated ATHEROSCLEROSIS. Many affected individuals develop malignant tumors, especially SARCOMA.
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Proto Oncogene Proteins c erbB 2
- cell surface protein-tyrosine kinase that is found to be overexpressed in significant number adenocarcinomas. It has extensive homology can heterodimerize EGF EPIDERMAL GROWTH FACTOR), 3 receptor (RECEPTOR, 3) and the 4 receptor. Activation of erbB-2 receptor occurs during heterodimer formation with a ligand-bound erbB family members. EC 2.7.11.-.