Balancing between Adaptive and Maladaptive Cellular Stress Responses in Peripheral Neuropathy.

Balancing between Adaptive and Maladaptive Cellular Stress Responses in Peripheral Neuropathy. Research Abstract Details 

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Balancing between Adaptive and Maladaptive Cellular Stress Responses in Peripheral Neuropathy. Abstract Text:

Mehrdad Khajavi,James R Lupski,

Point mutations in "myelin genes" result in a spectrum of inherited demyelinating neuropathies. The understanding of the pathomechanisms by which these mutations produce phenotypes remains limited. In this issue of Neuron, Wrabetz and colleagues report that the unfolded protein response (UPR) is responsible for demyelination in a Charcot-Marie-Tooth disease type 1B (CMT1B) mouse model. Deletion of the UPR mediator transcription factor CHOP completely rescues the motor deficit and ameliorates the neuropathy phenotype.

Balancing between Adaptive and Maladaptive Cellular Stress Responses in Peripheral Neuropathy. Publishing Authors By Initials

M Khajavi,JR Lupski,

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Balancing between Adaptive and Maladaptive Cellular Stress Responses in Peripheral Neuropathy. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Neuron

VOLUME: 57

Page Numbers: 329-30

Journal Abbreviation: Neuron

ISSN: 0896-6273

DAY: 7

MONTH: Feb

YEAR: 2008

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LANGUAGE: eng

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AFFILIATION: Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA.

Country: United States

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MEDLINETA: Neuron

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