Search Within
AB1576
Applied Filters:
Keyword:'AB1576'
Showing 1-13 of 13 results for "AB1576" within Papers
Zhiyong Liu et al.
The Journal of neuroscience : the official journal of the Society for Neuroscience, 32(19), 6600-6610 (2012-05-11)
Unlike nonmammalian vertebrates, mammals cannot convert inner ear cochlear supporting cells (SCs) into sensory hair cells (HCs) after damage, thus causing permanent deafness. Here, we achieved in vivo conversion of two SC subtypes, pillar cells (PCs) and Deiters' cells (DCs)
Lukas Rüttiger et al.
Proceedings of the National Academy of Sciences of the United States of America, 101(35), 12922-12927 (2004-08-26)
The large conductance voltage- and Ca2+-activated potassium (BK) channel has been suggested to play an important role in the signal transduction process of cochlear inner hair cells. BK channels have been shown to be composed of the pore-forming alpha-subunit coexpressed
Huzefa Rupawala et al.
Brain communications, 4(4), fcac192-fcac192 (2022-08-06)
In Alzheimer's disease, synapse loss causes memory and cognitive impairment. However, the mechanisms underlying synaptic degeneration in Alzheimer's disease are not well understood. In the hippocampus, alterations in the level of cysteine string protein alpha, a molecular co-chaperone at the
Erica L Gorenberg et al.
PLoS biology, 20(3), e3001590-e3001590 (2022-04-01)
Loss-of-function mutations in the depalmitoylating enzyme palmitoyl protein thioesterase 1 (PPT1) cause neuronal ceroid lipofuscinosis (NCL), a devastating neurodegenerative disease. The substrates of PPT1 are largely undescribed, posing a limitation on molecular dissection of disease mechanisms and therapeutic development. Here
Burak Uzay et al.
Cell reports, 42(2), 112042-112042 (2023-01-27)
Rapid release of neurotransmitters in synchrony with action potentials is considered a key hardwired property of synapses. Here, in glutamatergic synapses formed between induced human neurons, we show that action potential-dependent neurotransmitter release becomes progressively desynchronized as synapses mature and
Yong-Quan Zhang et al.
Biochimica et biophysica acta, 1842(11), 2136-2146 (2014-07-30)
Cysteine String Protein alpha (CSPα) is a palmitoylated, synaptic vesicle co-chaperone that is essential for neuroprotection. Two mutations in CSPα - L115R and L116Δ - cause adult neuronal ceroid lipofuscinosis (ANCL), a dominantly-inherited neurodegenerative disease. To elucidate the pathogenesis of
Evidence that the presynaptic vesicle protein CSPalpha is a key player in synaptic degeneration and protection in Alzheimer's disease.
Tiwari, SS; d'Orange, M; Troakes, C; Shurovi, BN; Engmann, O; Noble, W; Hortobagyi, T; Giese, KP
Molecular Brain null
Ching-Feng Chen et al.
Visual neuroscience, 39, E003-E003 (2022-05-12)
During the first postnatal week in rodents, cholinergic retinal waves initiate in starburst amacrine cells (SACs), propagating to retinal ganglion cells (RGCs) and visual centers, essential for visual circuit refinement. By modulating exocytosis in SACs, dynamic changes in the protein
Michael X Henderson et al.
Acta neuropathologica, 131(4), 621-637 (2015-12-15)
Neuronal ceroid lipofuscinoses (NCL) are a group of inherited neurodegenerative disorders with lysosomal pathology (CLN1-14). Recently, mutations in the DNAJC5/CLN4 gene, which encodes the presynaptic co-chaperone CSPα were shown to cause autosomal-dominant NCL. Although 14 NCL genes have been identified
Laura Caló et al.
Brain : a journal of neurology, 144(6), 1661-1669 (2021-03-25)
α-Synuclein aggregation at the synapse is an early event in Parkinson's disease and is associated with impaired striatal synaptic function and dopaminergic neuronal death. The cysteine string protein (CSPα) and α-synuclein have partially overlapping roles in maintaining synaptic function and
Bruno A Benitez et al.
Acta neuropathologica communications, 3, 73-73 (2015-11-28)
Autosomal dominant adult-onset neuronal ceroid lipofuscinosis (AD-ANCL) is a multisystem disease caused by mutations in the DNAJC5 gene. DNAJC5 encodes Cysteine String Protein-alpha (CSPα), a putative synaptic protein. AD-ANCL has been traditionally considered a lysosomal storage disease based on the
Attilio Iemolo et al.
NPJ Parkinson's disease, 9(1), 92-92 (2023-06-17)
Cognitive deficits, including working memory, and visuospatial deficits are common and debilitating in Parkinson's disease. α-synucleinopathy in the hippocampus and cortex is considered as the major risk factor. However, little is known about the progression and specific synaptic mechanisms underlying
Bruno A Benitez et al.
Scientific reports, 7(1), 6332-6332 (2017-07-26)
Mutations in the co- chaperone protein, CSPα, cause an autosomal dominant, adult-neuronal ceroid lipofuscinosis (AD-ANCL). The current understanding of CSPα function exclusively at the synapse fails to explain the autophagy-lysosome pathway (ALP) dysfunction in cells from AD-ANCL patients. Here, we
Page 1 of 1