anti-Charged Multivesicular Body Protein 2B (CHMP2B) Anticorps

Human Charged Multivesicular Body Protein 2B (CHMP2B) interaction partners

1. We directly link TDP-43 loss of function toxicity to two genes with rare amyotrophic lateral sclerosis and frontotemporal lobar degeneration-causing mutations, CHMP2B and ErbB4

2. these data indicate that the neuronal expression of human CHMP2B(intron5) in areas involved in motor and cognitive functions induces progressive motor alterations associated with dementia symptoms and with histopathological hallmarks reminiscent of both amyotrophic lateral sclerosis and frontotemporal dementia.

3. Endogenous TMEM106B was partly sequestered in CHMP2B-positive structures. SNP T185 was more associated with CHMP2B than SNP S185, and it enhanced neurotoxicity caused by CHMP2B(Intron5) compared to S185-expressing cells.

4. Study showed that mutant CHMP2B causes the pathological accumulation of endolysosomal components early in the frontotemporal dementia disease course

5. Protein kinase CK2 alpha is involved in the phosphorylation of the ESCRT-III subunits CHMP3 and CHMP2B, as well as of VPS4B/SKD1, an ATPase that mediates ESCRT-III disassembly.

6. Data indicate that knockdown of syntaxin 13 (syx13) further increased the cellular toxicity caused by muaant CHMP2B (CHMP2BIntron5) expression.

7. CHMP2B immunoreactivity was increased in the dorsal motor nucleus of the vagus nerve in Parkinson's disease and incidental Lewy body disease brains

8. These findings suggest that endosomal and autophagic pathway is associated with degradation or formation of alpha-synuclein aggregates in alpha-synucleinopathy.

9. Subjects with CHMP2B mutation show cognitive changes dominated by executive dysfunctions, years before they fulfil diagnostic criteria of FTD.

10. Direct link between disease-causing mutations and the cellular phenotype in cells originating from CHMP2B mutation patients with frontotemporal dementia.

11. This study provided a better understanding of the cellular pathogenesis of neurodegenerative diseases associated with various missense mutations of CHMP2B as well as endocytic defects.

12. CHMP2B polymerization scaffolds membranes in vivo represents a first step toward demonstrating its structural role during outward membrane deformation

13. recent advances in our understanding of the molecular basis of CHMP2B mutations indicate that the mechanisms involved may be broadly relevant to neurodegenerative processes.[review]

14. A novel heterozygous variant p.Ser194Leu (c.581C>T) is found in exon 6 of the CHMP2B gene in one male patient with pure frontotemporal lobar degeneration.

15. The results of this study confirmed that mutations in CHMP2B are not a common cause of frontotemporal lobar degeneration.

16. CHMP2B is required for spine growth. Taken together, these results demonstrate that a mutant ESCRT-III subunit linked to a human neurodegenerative disease can disrupt the normal pattern of spine development.

17. analysis of CHMP2B mutations in lower motor neuron predominant amyotrophic lateral sclerosis

18. The fusion of endosomes with lysosomes is required for neuronal function suggesting a pathogenic mechanism for frontotemporal dementia caused by CHMP2B mutations.

19. CHMP2B can be used as a reliable marker for GVD in neurons of the AD hippocampus.

20. there were no significant differences in the frequencies of the IVS4 + 44C/A, 1303C/A, 1254T/C and IVS15Ex16-16C/G polymorphism haplotypes in the patient cohorts (regardless of the degree of hepatic iron deposition) compared to the control cohort

Mouse (Murine) Charged Multivesicular Body Protein 2B (CHMP2B) interaction partners

1. Data show that the frontotemporal dementia (FTD)-causing C-terminal truncation of CHMP2B results in dendritic endolysosomal trafficking defects. Strikingly, we found that this trafficking defect could be rescued by the knockdown of the FTD risk factor Tmem106b.

2. The pro-apoptotic JNK scaffold protein POSH mediates CHMP2BIntron5-associated toxicity in animal models of frontotemporal dementia.

3. The presence of early microglial changes in our CHMP2B mutant mice indicates neuroinflammation may be a contributing factor to the neurodegeneration observed in FTD.

4. Study showed that mutant CHMP2B causes the pathological accumulation of endolysosomal components early in the frontotemporal dementia disease course

5. This study showing that CHMP2B resides at synapses and is involved in synaptic plasticity.

6. Forebrain-specific expression of frontotemporal dementia-associated mutant CHMP2B in mice causes several age-dependent neurodegenerative phenotypes, including social behavioral impairments.

7. Chmp2b is one of the most highly regulated cell-autonomous targets of Lbx1 in the embryonic mouse neural tube

8. These data describe the first mouse model of dementia caused by CHMP2B mutation and provide new insights into the mechanisms of CHMP2B-induced neurodegeneration