Endoplasmic reticulum stress in spinal and bulbar muscular atrophy

Spinal and bulbar muscular atrophy is an X-linked degenerative motor neuron disease caused by an abnormal expansion in the polyglutamine ...

These findings show that endoplasmic reticulum stress is activated in spinal cord motor neurons of SBMA mice and that this occurs well before ...

Endoplasmic reticulum stress in spinal and bulbar muscular atrophy ...

Endoplasmic reticulum stress in spinal and bulbar muscular atrophy: a potential target for therapy. by Karli Montague, Bilal Malik, Anna L Gray, ...

Endoplasmic reticulum stress in spinal and bulbar muscular atrophy ...

PDF | Spinal and bulbar muscular atrophy is an X-linked degenerative motor neuron disease caused by an abnormal expansion in the polyglutamine encoding.

Endoplasmic reticulum stress in spinal and bulbar muscular atrophy

Endoplasmic reticulum stress in spinal and bulbar muscular atrophy: a potential target for therapy ... Authors: Karli Montague; Bilal Malik; Anna L. Gray; Albert ...

Confounding Roles of ER Stress and the Unfolded Protein ... - MDPI

The X-linked muscular dystrophy (mdx) mouse is the most widely used animal model for the DMD. Analysis of skeletal muscle from multiple studies ...

ER stress in skeletal muscle remodeling and myopathies - FEBS Press

Accumulating evidence suggests that ER stress-induced UPR pathways play pivotal roles in the regulation of skeletal muscle mass and metabolic function in ...

Gene expression analysis reveals early dysregulation of disease ...

Spinal and bulbar muscular atrophy (SBMA) results from a CAG repeat expansion within the androgen receptor gene (AR).

Endoplasmic reticulum stress in spinal and bulbar muscular atrophy ...

Endoplasmic reticulum stress in spinal and bulbar muscular atrophy: a potential target for therapy ; Bilal Malik, Anna L. Gray, Albert R. La Spada, Michael G.

Unveiling synapse pathology in spinal bulbar muscular atrophy by ...

The results revealed the involvement of the pathology associated with synapses, epigenetics, and endoplasmic reticulum (ER) in SBMA.

Potential roles of the endoplasmic reticulum stress pathway in ...

The endoplasmic reticulum (ER) is a major organelle involved in protein quality control and cellular homeostasis. ER stress results from structural and ...

Spinal and bulbar muscular atrophy: From molecular pathogenesis ...

The dysfunction of the ubiquitin-proteasome pathway in SBMA is particularly striking, considering that skeletal muscle atrophy caused by various factors is ...

Mid1 is associated with androgen-dependent axonal vulnerability of ...

Spinal and bulbar muscular atrophy (SBMA) is an adult-onset hereditary neurodegenerative disease caused by the expansions of CAG repeats in ...

Potential roles of the endoplasmic reticulum stress pathway in ...

Neurons are particularly sensitive to the accumulation of misfolded proteins. Thus, the ER stress is involved in neurodegenerative diseases such ...

Endoplasmic reticulum stress in spinal and bulbar muscular atrophy

Endoplasmic reticulum stress in spinal and bulbar muscular atrophy: a potential target for therapy. Montague K;Malik B;Gray AL;La Spada AR;Hanna MG ...

Oxidative and endoplasmic reticulum stress interplay in sporadic ...

Here we show that spinal cord from patients with sporadic ALS showed signs of ER stress, such as increased levels of ER chaperones such as protein-disulfide ...

Deterioration of muscle force and contractile characteristics are early ...

The pathological hallmarks of the disease are selective loss of spinal and bulbar motor neurons, accompanied by weakness, atrophy and fasciculations of bulbar ...

The role of ubiquitination in spinal and bulbar muscular atrophy

Spinal and bulbar muscular atrophy (SBMA) is a neurodegenerative and neuromuscular genetic disease caused by the expansion of a polyglutamine-encoding CAG ...

Targeting ER stress/ER stress response in myopathies - ScienceDirect

Chronic ER stress has been observed in muscle biopsies of patients with congenital myotonic dystrophy type I: the atrophic muscles fibres showed the aberrant ...

[PDF] FUS is not dysregulated by the spinal bulbar muscular atrophy ...

Endoplasmic reticulum stress in spinal and bulbar muscular atrophy: a potential target for therapy · K. MontagueB. Malik +4 authors. L. Greensmith. Medicine ...