Results 21 to 30 of about 1,792 (176)
Herpesvirus Tegument Protein pUL37 Interacts with Dystonin/BPAG1 To Promote Capsid Transport on Microtubules during Egress [PDF]
ABSTRACT Herpes simplex virus 1 (HSV-1) is a neurotropic virus that travels long distances through cells using the microtubule network. Its 125-nm-diameter capsid is a large cargo which efficiently recruits molecular motors for movement. Upon entry, capsids reach the centrosome by minus-end-directed transport.
David Pasdeloup +4 more
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Hereditary sensory autonomic neuropathy type VI in the age of genetic testing
Background Hereditary sensory and autonomic neuropathy type VI (HSAN VI) is a rare recessive genetic disorder caused by mutations in the human dystonin (DST) gene.
Lekshmi Peringassery Sateesh +5 more
doaj +2 more sources
The Role of Neuronal Dystonin in Sensory Neuron Survival
The mouse dystonin gene (Dst; previously Bpag1) yields three tissue specific isoforms of a giant cytoskeletal linker protein, namely the skin isoform dystonin-e, neuronal isoform dystonin-a, and muscle isoform dystonin-b. Through alternative splicing events at the 5’ end of neuronal and muscle transcripts, three further isoforms with unique N-termini ...
Anisha Lynch‐Godrei
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One gene but different proteins and diseases: the complexity of dystonin and bullous pemphigoid antigen 1 [PDF]
AbstractSince the immunochemical identification of the bullous pemphigoid antigen 230 (BP230) as one of the major target autoantigens of bullous pemphigoid (BP) in 1981, our understanding of this protein has significantly increased. Cloning of its gene, development and characterization of animal models with engineered gene mutations or spontaneous ...
Kseniia Künzli +3 more
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Dystonin/BPAG1 Promotes Plus-End-Directed Transport of Herpes Simplex Virus 1 Capsids on Microtubules during Entry [PDF]
ABSTRACT During infection by herpes simplex virus 1 (HSV-1), the viral capsid is transported around the cytoplasm along the microtubule (MT) network. Although molecular motors have been implicated in this process, the composition of the molecular machinery required for efficient directional transport is unknown.
Marion McElwee +4 more
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Differential expression of dystonin in cancers of the breast.
Breast cancer affects women at relatively high frequency (1). We mined published microarray datasets (2, 3) to determine in an unbiased fashion and at the systems level genes most differentially expressed in the primary tumors of patients with breast cancer.
Shahan Mamoor
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New roles for an old giant: Novel interactions of the dystonin protein
Dystonin/Bpag1 is a large cytoskeletal plakin protein that contributes to the integrity and subcellular structure of various cell types. Loss of dystonin in mice results in neuromuscular dysfunction and early death in a mouse mutant called dystonia musculorum.
Kunal Bhanot
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Dystonin is a cytoskeletal linker protein whose loss-of-function in dystonia musculorum (dt) mice results in a hereditary sensory neuropathy with profound sensory ataxia. The dystonin gene (Dst) is exceptionally large (~400 kb) producing three giant neuronal dystonin isoforms (>600 kDa) through alternative splicing, namely dystonin-a1, -a2, and -a3.
Andrew Ferrier
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