The neurochaperonopathies: Anomalies of the chaperone system with pathogenic effects in neurodegenerative and neuromuscular disorders

F Scalia, AM Vitale, R Santonocito… - Applied Sciences, 2021 - mdpi.com
The chaperone (or chaperoning) system (CS) constitutes molecular chaperones, co-
chaperones, and chaperone co-factors, interactors and receptors, and its canonical role is …

Impaired mitochondrial mobility in Charcot-Marie-Tooth disease

CR Schiavon, GS Shadel, U Manor - Frontiers in Cell and …, 2021 - frontiersin.org
Charcot-Marie-Tooth (CMT) disease is a progressive, peripheral neuropathy and the most
commonly inherited neurological disorder. Clinical manifestations of CMT mutations are …

KIF1Bβ mutations detected in hereditary neuropathy impair IGF1R transport and axon growth

F Xu, H Takahashi, Y Tanaka, S Ichinose, S Niwa… - Journal of Cell …, 2018 - rupress.org
KIF1Bβ is a kinesin-3 family anterograde motor protein essential for neuronal development,
viability, and function. KIF1Bβ mutations have previously been reported in a limited number …

Mutations in heat shock protein beta-1 (HSPB1) are associated with a range of clinical phenotypes related to different patterns of motor neuron dysfunction: A case …

M Katz, M Davis, FC Garton, R Henderson… - Journal of the …, 2020 - Elsevier
Background Heat shock protein beta-1 (HSPB1) is a ubiquitously expressed molecular
chaperone that is important in protecting cells against cellular injury. Mutations in this protein …

Comparison of direct sequencing, real-time PCR-high resolution melt (PCR-HRM) and PCR-restriction fragment length polymorphism (PCR-RFLP) analysis for …

WY Fong, CC Ho, WT Poon - Diagnostics, 2017 - mdpi.com
Thiopurine intolerance and treatment-related toxicity, such as fatal myelosuppression, is
related to non-function genetic variants encoding thiopurine S-methyltransferase (TPMT) …

Novel Tetra-Primer ARMS-PCR Assays for Thiopurine Intolerance Susceptibility Mutations NUDT15 c.415C>T and TPMT c.719A>G (TPMT*3C) in East Asians

CC Ho, WY Fong, YH Lee, WT Poon - Genes, 2017 - mdpi.com
Thiopurines are clinically useful in the management of diverse immunological and malignant
conditions. Nevertheless, these purine analogues can cause lethal myelosuppression …

A novel method that allows SNP discrimination with 160: 1 ratio for biosensors based on DNA-DNA hybridization

SB Nimse, KS Song, SD Warkad, T Kim - Biosensors, 2021 - mdpi.com
Highly sensitive (high SBR) and highly specific (high SNP discrimination ratio) DNA
hybridization is essential for a biosensor with clinical application. Herein, we propose a …

GNPTAB c.2404C > T nonsense mutation in a patient with mucolipidosis III alpha/beta: a case report

CC Ho, LLY Tsung, KT Liu, WT Poon - BMC Medical Genetics, 2018 - Springer
Background Mucolipidosis alpha/beta is an inborn error of metabolism characterized by
deficiency of GlcNAc-1-phosphotransferase, in which essential alpha/beta subunits are …

[HTML][HTML] Мутация в гене CREBBP у девочки с синдромом ходьбы на носках: клинический случай

Д Помарино, А Трен, К Ростази… - Вопросы современной …, 2021 - cyberleninka.ru
Обоснование. Патогенные варианты гена CREBBP выявлены у пациентов с
синдромами Рубинштейна-Тейби и Менке-Хеннекама, однако симптом ходьбы на …

Mutation in the CREBBP gene in the girl with toe walking syndrome: clinical case

D Pomarino, A Thren, JR Thren, K Rostasy… - Current …, 2021 - vsp.spr-journal.ru
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Мутация в гене CREBBP у девочки с синдромом ходьбы на носках: клинический случай …