Amyotrophic lateral sclerosis (ALS) is probably biphasic. An initial trigger(s) is followed by a terminal cascade coinciding with the onset of neurological deficits. The terminal cascade involves interactive multifactorial pathogenic mechanisms. Aging must play a crucial role leading to multiple defective...
To detect apoptotic cells in the nasopharynx, the terminal deoxynucleotidyl transferase biotin-dUTP nick end labelling (TUNEL) assay was performed according to the manufacturer’s instructions (12156792910, Merck). Imaging and morphometric analyses Immunofluorescence images were acquired using the LSM800 or...
In this study, we demonstrate that the 12.5kDa product of cystatin C is formed by degradation of the first eight N-terminal residues. Moreover, such a degradation is not specific in the cerebrospinal fluid of multiple sclerosis, but rather is given by an inappropriate sample storage at −20...
Homo-oligomerization of ALS2 through Its Unique Carboxyl-terminal Regions Is Essential for the ALS2-associated Rab5 Guanine Nucleotide Exchange Activity an... Mutations in the ALS2 gene have been known to account for a juvenile recessive form of amyotrophic lateral sclerosis (ALS2), a rare juven...
synaptic transmission is disrupted as a result of presynaptic terminal structure alterations in the Purkinje cell-DCN neuron in bothLESandLEHetanimals. These findings suggest that myelination deficits alter axonal and presynaptic terminal structures, disrupt AP propagation, and obstruct functional neurotransmis...
Axonal degeneration in multiple sclerosis: the mitochondrial hypothesis Curr. Neurol. Neurosci. Rep., 9 (2009), pp. 411-417 CrossrefView in ScopusGoogle Scholar [12] L.M. Dejean, S. Martinez-Caballero, K.W. Kinnally Is MAC the knife that cuts cytochrome c from mitochondria during apoptosis...
TDP-43 Is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity. Non-amyloid, ubiquitinated cytoplasmic inclusions containing TDP-43 and its C-terminal fragments are pathological hallmarks of amyotrophic lateral sclerosi... Johnson,Brian...
Suppression of tumorigenicity by the wild-type tuberous sclerosis 2 (Tsc2) gene and its C-terminal region. The Tsc2 gene, which is mutationally inactivated in the germ line of some families with tuberous sclerosis, encodes a large, membrane-associated GTPase act... F,Jin,R,... - 《Procee...
As we did for the wild-type UCH-L1, we generated an N-terminal fusions of the BioID1 and BioID2 biotin ligases that behave similarly in proteomic assays but differ by mass3,16,17. We observed a similar expression and catalytic activity with all fusions and selected the BioID2 constructs...
Zn, N-terminal zinc-binding domain (residues 20–86). Pro, proline-rich region (residues 86–148). The C-terminal helical domain can be subdivided into two predicted α-helices H1 (150–167) and H2 (173–195). b Schematic domain organization of representative SSSCA1 orthologs. Zn, N-...