Редактирование: BICD2 (раздел)

==Publications==

{{medline-entry
|title=A novel mouse model with impaired dynein/dynactin function develops amyotrophic lateral sclerosis (ALS)-like features in motor neurons and improves lifespan in [[SOD1]]-ALS mice.
|pubmed-url=https://pubmed.ncbi.nlm.nih.gov/18579581
|abstract=Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative condition characterized by progressive motor neuron degeneration and muscle paralysis. Genetic evidence from man and mouse has indicated that mutations in the dynein/dynactin motor complex are correlated with motor neuron degeneration. In this study, we have generated transgenic mice with neuron-specific expression of Bicaudal D2 N-terminus ([[BICD2]]-N) to chronically impair dynein/dynactin function. Motor neurons expressing [[BICD2]]-N showed accumulation of dynein and dynactin in the cell body, Golgi fragmentation and several signs of impaired retrograde trafficking: the appearance of giant neurofilament swellings in the proximal axon, reduced retrograde labelling by tracer injected in the muscle and delayed expression of the injury transcription factor [[ATF3]] after axon transection. Despite these abnormalities, [[BICD2]]-N mice did not develop signs of motor neuron degeneration and motor abnormalities. Interestingly, the [[BICD2]]-N transgene increased lifespan in 'low copy' [[SOD1]]-G93A ALS transgenic mice. Our findings indicate that impaired dynein/dynactin function can explain several pathological features observed in ALS patients, but may be beneficial in some forms of ALS.
|mesh-terms=* Amyotrophic Lateral Sclerosis
* Animals
* Biological Transport
* Carrier Proteins
* Cells, Cultured
* Disease Models, Animal
* Dynactin Complex
* Dyneins
* Female
* Gene Expression
* Golgi Apparatus
* Humans
* Life Expectancy
* Male
* Membrane Proteins
* Mice
* Mice, Transgenic
* Microtubule-Associated Proteins
* Motor Neurons
* Rats
* Superoxide Dismutase
* Superoxide Dismutase-1
* Survival

|full-text-url=https://sci-hub.do/10.1093/hmg/ddn182
}}