نتایج جستجو برای: pantothenate kinase associated neurodegeneration

تعداد نتایج: 1714897  

Journal: :The Journal of biological chemistry 2003
Thomas Kupke Pilar Hernández-Acosta Francisco A Culiáñez-Macià

Coenzyme A is required for many synthetic and degradative reactions in intermediary metabolism and is the principal acyl carrier in prokaryotic and eukaryotic cells. Coenzyme A is synthesized in five steps from pantothenate, and recently the CoaA biosynthetic genes in bacteria and human have all been identified and characterized. Coenzyme A biosynthesis in plants is not fully understood, and to...

Objective(s): Neurodegeneration is an outcome of Methamphetamine (METH) abuse. Studies have emphasized on the neuroprotective properties of lithium. The current study is designed towards evaluating the role of Akt-1/GSK3 and CREB-BDNF signaling pathways in mediating lithium neuroprotection against METH-induced neurodegeneration in rats. Materials and ...

2014
Shigeru Chohnan Misa Murase Kota Kurikawa Kodai Higashi Yuta Ogata

Pantothenol is a provitamin of pantothenic acid (vitamin B5) that is widely used in healthcare and cosmetic products. This analog of pantothenate has been shown to markedly inhibit the phosphorylation activity of the prokaryotic type II pantothenate kinase of Staphylococcus aureus, which catalyzes the first step of the coenzyme A biosynthetic pathway. Since type II enzymes are found exclusively...

Journal: :Journal of magnetic resonance imaging : JMRI 2010
Jerzy Szumowski Erhan Bas Kirsten Gaarder Erwin Schwarz Deniz Erdogmus Susan Hayflick

PURPOSE To investigate spatial distribution of iron accumulation in the globus pallidus (GP) in patients with Hallevorden-Spatz syndrome (HSS) using phase imaging. We compared sensitivity of a phase imaging technique to relaxation rate measurement methods (R1,R2,R2*) for iron quantification. MATERIALS AND METHODS R1, R2, and R2* were measured in GP structure of the brain of eight pantothenate...

Journal: :Molecular biology and evolution 2004
Ulrich Genschel

Coenzyme A (CoA) holds a central position in cellular metabolism and therefore can be assumed to be an ancient molecule. Starting from the known E. coli and human enzymes required for the biosynthesis of CoA, phylogenetic profiles and chromosomal proximity methods enabled an almost complete reconstruction of archaeal CoA biosynthesis. This includes the identification of strong candidates for ar...

2016
C. E. Arber A. Li H. Houlden S. Wray

Neurodegeneration with brain iron accumulation (NBIA) is a group of disorders characterized by dystonia, parkinsonism and spasticity. Iron accumulates in the basal ganglia and may be accompanied by Lewy bodies, axonal swellings and hyperphosphorylated tau depending on NBIA subtype. Mutations in 10 genes have been associated with NBIA that include Ceruloplasmin (Cp) and ferritin light chain (FTL...

Journal: :Vojnosanitetski pregled 2014
Dragan Krstić Jadranka Antonijević Željko Špirić

INTRODUCTION Wilson's disease is typically manifested in two clinical forms, neurological and hepatic and in rare cases it starts with psychiatric symptoms exclusively. We presented a rare atypical case of Wilson's disease with psy- chotic onset. CASE REPORT A 22-year-old male patient was initially presented with predominant signs and symptoms of psychiatric disorder and then later with the d...

Journal: :Human molecular genetics 2005
Yien-Ming Kuo Jacque L Duncan Shawn K Westaway Haidong Yang George Nune Eugene Yujun Xu Susan J Hayflick Jane Gitschier

Pantothenate kinase-associated neurodegeneration (PKAN, formerly known as Hallervorden-Spatz syndrome) is a rare but devastating neurodegenerative disorder, resulting from an inherited defect in coenzyme A biosynthesis. As pathology in the human condition is limited to the central nervous system, specifically the retina and globus pallidus, we have generated a mouse knock-out of the orthologous...

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