GANGLIOSIDE GM2
\ɡˈaŋɡlɪˌɒsa͡ɪd d͡ʒˌiːˈɛm tˈuː], \ɡˈaŋɡlɪˌɒsaɪd dʒˌiːˈɛm tˈuː], \ɡ_ˈa_ŋ_ɡ_l_ɪ__ˌɒ_s_aɪ_d dʒ_ˌiː__ˈɛ_m t_ˈuː]\
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A glycosphingolipid that accumulates due to a deficiency of hexosaminidase A or B (BETA-N-ACETYLHEXOSAMINIDASE), or GM2 activator protein, resulting in GANGLIOSIDOSES, heredity metabolic disorders that include TAY-SACHS DISEASE and SANDHOFF DISEASE.
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Word of the day
Procollagen Proline Dioxygenase
- mixed-function oxygenase that catalyzes hydroxylation prolyl-glycyl-containing-peptide, usually in protocollagen, hydroxyprolylglycyl-peptide. The enzyme utilizes molecular oxygen with a concomitant oxidative decarboxylation of 2-oxoglutarate to succinate. EC 1.14.11.2.