Recombinant Mouse Dystonin (Dst), partial

1. The present study identified novel mutant mice with a mild dt phenotype of sensory neurodegeneration and movement disorder. PMID: 27693510
2. oligodendrocytes do not have an intrinsic requirement for neuronal dystonin for differentiation and myelination. PMID: 26886550
3. Novel Dst(Gt) mice showed that a loss-of-function mutation in the actin-binding domain-containing Dystonin isoforms led to typical dystonia musculorum phenotypes PMID: 25195653
4. In the C2.7 cell line, knockdown of BPAG1a/b had no major effect on the organization of microtubules and microfilaments, but negatively affected endocytosis and Golgi apparatus structure and decreased cell migration but not cell adhesion. PMID: 25244344
5. Restoration of dystonin-a2 expression in the nervous system prevented the disorganization of organelle membranes and microtubule networks in a mouse model of hereditary sensory and autonomic neuropathy. PMID: 24381311
6. This study provides insight into the mechanism of dt neuropathology and proposes a role for dystonin-a2 as a mediator of normal ER structure and function. PMID: 22190742
7. novel functions of the dystonin-a2 isoform in mediating microtubule stability, Golgi organization, and flux through the secretory pathway. PMID: 22412020
8. Microtubule associated protein 1B, a microtubule stabilizing protein, and clathrin heavy chain, the major component of the clathrin triskelion, were identified as interaction partners for dystonin-a PMID: 21936565
9. dystonin does not significantly influence the structural organization of cardiac muscle fibers during early postnatal development PMID: 20209123
10. BPAG1-b was detectable in vitro and in vivo as a high molecular mass protein in striated and heart muscle cells, co-localizing with alpha-actinin-2 and partially with the cytolinker plectin as well as with the intermediate filament protein desmin. PMID: 19932097
11. BPAG1 has three kinds of cytoskeletal binding domains and seems to play an important role in linking the different types of cytoskeletons. PMID: 11751855
12. Transcripts for both neural isoforms of Bpag1 (a1 and a2) were detected in optic nerves and spinal cords of wild-type mice. PMID: 12111805
13. Bpag1 may be involved in the maintenance rather than the establishment of muscle cell architecture. Cytoskeletal disruptions are seen in muscle of postnatal dystonia musculorum mice. Early phases of myogenic differentiation occur independently of Bpag1. PMID: 12190986
14. BPAG1n4 is a novel neuronal isoform which plays an essential role in retrograde axonal transport in sensory neurons. PMID: 14581450
15. The overall structure and subcellular localizations of Bpag1 indicate possible functions in nuclear envelope structuring, nuclear tethering, and organization of membranous structures surrounding the nucleus. PMID: 16289082
16. These data together suggest that the survival of vagal and glossopharyngeal sensory neurons is dependent upon dystonin. PMID: 16289886
17. Alterations likely result in reduced or absent dystonin/Bpag1 protein levels. Thus, distinct genetic defects lead to a common outcome of reduced transcript expression causing the same phenotype in multiple dt alleles. PMID: 16341670
18. Dystonin/Bpag1 is not essential for microtubule network assembly since it is intact in short-term cultures of sensory neurons. Dystonin/Bpag1 is not an essential part of the dynein motor complex for mitochondrial transport. PMID: 16374799
19. we characterized these N-terminal isoforms and evaluated the influence of these unique N-terminal sequences to the actin-binding properties. The N-terminal region of isoform 1 is very short and did not affect the property of the ABD that followed it. PMID: 16797530
20. These data together suggest that dystonin is required for the innervation and development of fungiform papillae and taste buds. PMID: 17156752
21. retrolinkin acts with BPAG1n4 to specifically regulate retrograde axonal transport PMID: 17287360
22. Elucidating the roles of dystonin most relevant to neuronal function and survival should help to shed light on some of the common mechanisms underlying neurodegeneration. PMID: 17849487
23. These data together suggest that dystonin is necessary for survival of nociceptors but not proprioceptors in the trigeminal nervous system. PMID: 18619576
24. Defects in Dystonin mutant mice, as demonstrated here, may ultimately result in pathogenesis involving ER dysfunction and contribute significantly to the dt phenotype. PMID: 18638474

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KEGG: mmu:13518

STRING: 10090.ENSMUSP00000095392

UniGene: Mm.336625