Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0002736 (
amyotrophic lateral sclerosis
)
19,048
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
(1) Background:
Amyotrophic lateral sclerosis
(
ALS
) and frontotemporal dementia (FTD) are neurodegenerative disorders with an overlap in clinical presentation and neuropathology. Common and differential mechanisms leading to protein expression changes and neurodegeneration in
ALS
and FTD were studied trough a deep neuroproteome mapping of the spinal cord. (2) Methods: A liquid chromatography tandem mass spectrometry (LC-MS/MS) analysis of the spinal cord from
ALS
-TAR DNA-binding protein 43 (
TDP-43
) subjects, ubiquitin-positive frontotemporal lobar degeneration (FTLD-U) subjects and controls without neurodegenerative disease was performed. (3) Results: 281 differentially expressed proteins were detected among
ALS
versus controls, while 52 proteins were dysregulated among FTLD-U versus controls. Thirty-three differential proteins were shared between both syndromes. The resulting data was subjected to network-driven proteomics analysis, revealing mitochondrial dysfunction and metabolic impairment, both for
ALS
and FTLD-U that could be validated through the confirmation of expression levels changes of the Prohibitin (
PHB
) complex. (4) Conclusions:
ALS
-TDP-43 and FTLD-U share molecular and functional alterations, although part of the proteostatic impairment is region- and disease-specific. We have confirmed the involvement of specific proteins previously associated with
ALS
(Galectin 2 (
LGALS3
), Transthyretin (
TTR
), Protein S100-A6 (
S100A6
), and Protein S100-A11 (
S100A11
)) and have shown the involvement of proteins not previously described in the
ALS
context (Methanethiol oxidase (
SELENBP1
), Peptidyl-prolyl cis-trans isomerase NIMA-interacting 1 (
PIN-1
),
Calcyclin-binding protein
(
CACYBP
) and Rho-associated protein kinase 2 (
ROCK2
)).
...
PMID:Neuroanatomical Quantitative Proteomics Reveals Common Pathogenic Biological Routes between Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia (FTD). 3057 65
The S100A6 protein is present in different mammalian cells and tissues including the brain. It binds Ca
2+
and Zn
2+
and interacts with many target proteins/ligands. The best characterized ligands of S100A6, expressed at high level in the brain, include
CacyBP
/SIP and Sgt1. Research concerning the functional role of S100A6 and these two ligands indicates that they are involved in various signaling pathways that regulate cell proliferation, differentiation, cytoskeletal organization, and others. In this review, we focused on the expression/localization of these proteins in the brain and on their possible role in neurodegenerative diseases. Published results demonstrate that S100A6,
CacyBP
/SIP, and Sgt1 are expressed in various brain structures and in the spinal cord and can be found in different cell types including neurons and astrocytes. When it comes to their possible involvement in nervous system pathology, it is evident that their expression/level and/or subcellular localization is changed when compared to normal conditions. Among diseases in which such changes have been observed are Alzheimer's disease (AD),
amyotrophic lateral sclerosis
(
ALS
), epileptogenesis, Parkinson's disease (PD), Huntington's disease (HD), and others.
...
PMID:S100A6 and Its Brain Ligands in Neurodegenerative Disorders. 3249 24
