نتایج جستجو برای: metachromatic leukodystrophy

تعداد نتایج: 3076  

Journal: :International Journal of Case Reports and Images 2015

Journal: :Annales de biologie clinique 2010
Ilhem Barboura Salima Ferchichi Azza Dandana Zaineb Jaidane Souhaira Ben Khelifa Hinda Chahed Rachida Ben Mansour Saber Chebel Irène Maire Abdelhedi Miled

Scholz's disease or metachromatic leukodystrophy (MLD) is a lysosomal storage disease caused by a deficiency in arylsulfatase A (ARSA: EC 3.1.6.8). This enzyme is responsible for the degradation of sulfatides commonly called cerebroside-3-sulfate or 3-O-sulfogalactosylcéramide in galactocérébroside and sulfate. The success of hydrolysis of these sphingolipids by ARSA necessarily depends on the ...

Journal: :Value in Health 2022

MLD is an ultra-rare neurodegenerative disease leading to motor and cognitive decline premature death. Prior the introduction of atidarsagene autotemcel (arsa-cel), treatment consisted only best supportive care. When patients are treated pre-symptomatically, arsa-cel has potential prevent or slow progression enable children have improved quality life. In absence newborn screening (NBS), pre-sym...

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