Substrate reduction therapy

Substrate reduction therapy

Бусад хувилбаруудыг харуулах (1)

The glycosphingolipidoses are a family of storage diseases that arise due to incomplete catabolism of glycosphingolipids (GSLs) in the lysosome (Wraith, 2002). The majority are autosomal recessive disorders and result from mutations in the genes that encode the catabolic enzymes of the lysosome (Win...

Бүрэн тодорхойлолт

Номзүйн дэлгэрэнгүй
Үндсэн зохиолчид:	Platt, F, Butters, T
Формат:	Book section
Хэвлэсэн:	Springer US 2007

Ижил төстэй зүйлс

Substrate reduction therapy in mouse models of the glycosphingolipidoses.
-н: Platt, F, зэрэг
Хэвлэсэн: (2003)

Substrate reduction therapy.
-н: Platt, F, зэрэг
Хэвлэсэн: (2008)

Substrate reduction therapy for glycosphingolipid storage disorders.
-н: Lachmann, R, зэрэг
Хэвлэсэн: (2001)

Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease.
-н: Andersson, U, зэрэг
Хэвлэсэн: (2004)

Substrate deprivation: a new therapeutic approach for the glycosphingolipid lysosomal storage diseases.
-н: Platt, F, зэрэг
Хэвлэсэн: (2000)

Beneficial effects of substrate reduction therapy in a mouse model of GM1 gangliosidosis.
-н: Elliot-Smith, E, зэрэг
Хэвлэсэн: (2008)

Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease
-н: Ulrika Andersson, зэрэг
Хэвлэсэн: (2004-08-01)

Substrate reduction therapy in the infantile form of Tay-Sachs disease.
-н: Bembi, B, зэрэг
Хэвлэсэн: (2006)

Substrate reduction therapy reduces brain ganglioside GM2 in neonatal Sandhoff disease mice
-н: Baek, R, зэрэг
Хэвлэсэн: (2005)

Lucerastat, an iminosugar for substrate reduction therapy in Fabry disease: preclinical evidence
-н: Priestman, D, зэрэг
Хэвлэсэн: (2017)

Lucerastat, an iminosugar for substrate reduction therapy in Fabry Disease: Preclinical evidence
-н: Welford, R, зэрэг
Хэвлэсэн: (2017)

Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation.
-н: Jeyakumar, M, зэрэг
Хэвлэсэн: (2001)

Substrate reduction therapy in Sandhoff disease: Evidence for improvement in nervous function in patients treated with miglustat
-н: Lachmann, R, зэрэг
Хэвлэсэн: (2006)

Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
-н: Jeyakumar, M, зэрэг
Хэвлэсэн: (2002)

Imino sugar therapy for type 1 Gaucher disease.
-н: Priestman, D, зэрэг
Хэвлэсэн: (2000)

Substrate reduction therapy for inborn errors of metabolism
-н: Yue, WW, зэрэг
Хэвлэсэн: (2019)

Inhibition of substrate synthesis as a strategy for glycolipid lysosomal storage disease therapy.
-н: Platt, F, зэрэг
Хэвлэсэн: (2001)

Miglustat - A viewpoint
-н: Platt, F, зэрэг
Хэвлэсэн: (2003)

Glycolipid depletion in antimicrobial therapy.
-н: Svensson, M, зэрэг
Хэвлэсэн: (2003)

Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice
-н: J.L. Kasperzyk, зэрэг
Хэвлэсэн: (2005-04-01)

Eliglustat substrate reduction therapy in children with Gaucher disease type 1
-н: Noor Ul Ain, зэрэг
Хэвлэсэн: (2025-02-01)

Effects of substrate deprivation therapy on brain lipids in neonatal GML storage disease mice
-н: Kasperzyk, J, зэрэг
Хэвлэсэн: (2003)

Iminosugar inhibitors for treating the lysosomal glycosphingolipidoses
-н: Butters, T, зэрэг
Хэвлэсэн: (2005)

Therapeutic applications of imino sugars in lysosomal storage disorders.
-н: Butters, T, зэрэг
Хэвлэсэн: (2003)

Imino sugar inhibitors for treating the lysosomal glycosphingolipidoses.
-н: Butters, T, зэрэг
Хэвлэсэн: (2005)

New therapeutics for the treatment of glycosphingolipid lysosomal storage diseases.
-н: Butters, T, зэрэг
Хэвлэсэн: (2003)

Inhibition of glycosphingolipid biosynthesis: application to lysosomal storage disorders.
-н: Butters, T, зэрэг
Хэвлэсэн: (2000)

Genetic Substrate Reduction Therapy: A Promising Approach for Lysosomal Storage Disorders
-н: Maria Francisca Coutinho, зэрэг
Хэвлэсэн: (2016-11-01)

"Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice" (vol 46, pg 744, 2004)
-н: Kasperzyk, J, зэрэг
Хэвлэсэн: (2005)

Substrate deprivation therapy reduces brain ganglioside content and GM1 in neonatal storage disease mice
-н: Kasperzyk, J, зэрэг
Хэвлэсэн: (2002)

Glycolipids in cell biology and medicine - Introduction
-н: Dwek, R, зэрэг
Хэвлэсэн: (2003)

Introduction
-н: Dwek, R, зэрэг
Хэвлэсэн: (2003)

Structure-based inhibitor design for substrate reduction therapy towards primary hyperoxaluria and galactosaemia
-н: Mackinnon, S
Хэвлэсэн: (2019)

Substrate reduction augments the efficacy of enzyme therapy in a mouse model of Fabry disease.
-н: John Marshall, зэрэг
Хэвлэсэн: (2010-11-01)

Cellular and biochemical response to chaperone versus substrate reduction therapies in neuropathic Gaucher disease
-н: Margarita M. Ivanova, зэрэг
Хэвлэсэн: (2021-01-01)

Cellular and biochemical response to chaperone versus substrate reduction therapies in neuropathic Gaucher disease.
-н: Margarita M Ivanova, зэрэг
Хэвлэсэн: (2021-01-01)

Glycosylation: disease targets and therapy.
-н: Zitzmann, N, зэрэг
Хэвлэсэн: (2005)

Effect of Substrate Reduction Therapy in Comparison to Enzyme Replacement Therapy on Immune Aspects and Bone Involvement in Gaucher Disease
-н: Renuka P. Limgala, зэрэг
Хэвлэсэн: (2020-03-01)

Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis.
-н: Cox, T, зэрэг
Хэвлэсэн: (2000)

Erratum: Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice (Journal of Lipid Research (April 2004) 46 (744-751))
-н: Kasperzyk, J, зэрэг
Хэвлэсэн: (2005)