A5085636073- Lysosomal Storage Disorders Research
- MicroRNA in disease regulation
- Cancer-related molecular mechanisms research
- Cytomegalovirus and herpesvirus research
- RNA Research and Splicing
- Circular RNAs in diseases
- RNA modifications and cancer
- Sarcoma Diagnosis and Treatment
- Virus-based gene therapy research
- Neurogenesis and neuroplasticity mechanisms
- Neurogenetic and Muscular Disorders Research
- Cellular transport and secretion
- Neurological Disease Mechanisms and Treatments
- Herpesvirus Infections and Treatments
- Barrier Structure and Function Studies
- Glycosylation and Glycoproteins Research
- Autoimmune and Inflammatory Disorders Research
- Muscle Physiology and Disorders
- Virology and Viral Diseases
- Retinal Development and Disorders
- Neural Networks and Reservoir Computing
- RNA regulation and disease
- Bone Tumor Diagnosis and Treatments
- Platelet Disorders and Treatments
- Neuroinflammation and Neurodegeneration Mechanisms
Xinjiang Medical University
2025
First Affiliated Hospital of Xinjiang Medical University
2025
Hunan Cancer Hospital
2020-2024
Central South University
2014-2024
Xidian University
2022
Virginia Tech
2022
Hunan Normal University
2019-2020
Institute of Subtropical Agriculture
2019
Changsha Medical University
2019
Sanofi (United States)
2018-2019
Central nervous system–directed antisense therapy ameliorates symptoms in a severe neuromuscular disorder mice.
Spinal muscular atrophy (SMA) is a neuromuscular disease caused by deficiency of survival motor neuron (SMN) due to mutations in the SMN1 gene. In this study, an adeno-associated virus (AAV) vector expressing human SMN (AAV8-hSMN) was injected at birth into CNS mice modeling SMA. Western blot analysis showed that these injections resulted widespread expression throughout spinal cord, and translated robust improvement skeletal muscle physiology, including increased myofiber size improved...
Mutations of GBA1 , the gene encoding glucocerebrosidase, represent a common genetic risk factor for developing synucleinopathies Parkinson disease (PD) and dementia with Lewy bodies. PD patients or without mutations also exhibit lower enzymatic levels glucocerebrosidase in central nervous system (CNS), suggesting possible link between enzyme development disease. Previously, we have shown that early treatment can modulate α-synuclein aggregation presymptomatic mouse model Gaucher-related...
The intestinal epithelial barrier, which works as the first line of defense between luminal environment and host, once destroyed, it will cause serious inflammation or other diseases. Tight junctions (TJs) play a vital role to maintain integrity barrier. Lipopolysaccharide (LPS), one most important inflammatory factors downregulate specific TJ proteins including Occludin Claudin-1 impair Betaine has excellent anti-inflammatory activity but whether betaine any effect on proteins, particularly...
To explore the association between blood pressure (BP) metrics and early neurological deterioration of ischemic origin (ENDi) in patients with mild stroke large vessel occlusion (LVO) undergoing best medical management (BMM). Data were collected from consecutive LVO treated BMM January 2019 to December 2023. Admission systolic (SBP), diastolic (DBP), mean arterial (MAP) 24-h SBP variability calculated. ENDi was defined as an National Institutes Health Stroke Scale (NIHSS) score increase ≥ 4...
Classical late infantile neuronal ceroid lipofuscinosis (cLINCL) is a lysosomal storage disorder caused by mutations in CLN2 , which encodes tripeptidyl peptidase I (TPP1). Lack of TPP1 results accumulation autofluorescent material and curvilinear bodies cells throughout the CNS, leading to progressive neurodegeneration death typically childhood. In this study, we injected adeno-associated virus (AAV) vectors containing human cDNA into brains −/− mice determine therapeutic efficacy. AAV2 CU...
Spinal muscular atrophy (SMA) is a neuromuscular disease caused by mutations in survival motor neuron 1 (SMN1). Previously, we showed that central nervous system (CNS) delivery of an adeno-associated viral (AAV) vector encoding SMN1 produced significant improvements mouse model SMA. Here, performed dose-response study SMA mice to determine the levels SMN spinal cord necessary for efficacy, and measured efficiency transduction after intrathecal pigs nonhuman primates (NHPs). CNS injections...
Abstract Cells that express the NG2 proteoglycan (NG2+ cells) constitute a large glial population in normal mature rodent brain. They can differentiate into oligodendrocytes but are distinct from oligodendrocytes, astrocytes, microglia, and neurons. Changes NG2+ cells were examined kainic acid‐induced excitotoxic lesions of hippocampus, relationship between reactive astrocytes microglia was investigated 1 90 days after lesioning. Two types with altered morphology increased immunoreactivity...
Niemann-Pick A disease (NPA) is a fatal lysosomal storage disorder caused by deficiency in acid sphingomyelinase (ASM) activity. The lack of functional ASM results cellular accumulation sphingomyelin and cholesterol within distended lysosomes throughout the brain. In this study, we investigated potential AAV-mediated expression to correct brain pathology an knockout (ASMKO) mouse model NPA. An AAV serotype 2 vector encoding human (AAV2-hASM) was injected directly into adult ASMKO hippocampus...
Niemann-Pick type A disease is a lysosomal storage disorder caused by deficiency in acid sphingomyelinase (ASM) activity. Previously we showed that pathology the ASM knockout (ASMKO) mouse brain can be corrected adeno-associated virus serotype 2 (AAV2)-mediated gene transfer. The present experiment compared relative therapeutic efficacy of different recombinant AAV vectors (1, 2, 5, 7, and 8) using histological, biochemical, behavioral endpoints. In addition, evaluated use deep cerebellar...
The dysmyelinating mutant jimpy ( jp ) arises from a point mutation in the mouse gene encoding proteolipid protein and is characterized by severe dysmyelination attributable to oligodendrocyte death. This was used investigate regulation of progenitor proliferation postnatal spinal cord. At day 18, cord contained three- eightfold greater number proliferating cells than did wild-type wt Increased accompanied twofold increase cells. Semiquantitative reverse transcriptase-PCR revealed no change...
Abstract Glial cells that express the NG2 proteoglycan (NG2 + cells) are considered to be oligodendrocyte progenitors (OPCs) in central nervous system (CNS), based on their ability give rise mature oligodendrocytes vitro. To understand how dysmyelinated conditions influence OPC proliferation and differentiation, we studied differentiation of OPCs vivo shiverer mutant (shi), which do not form compact myelin due a deletion basic protein gene. Acute bromodeoxyuridine (BrdU) labeling studies...
Classical late infantile neuronal ceroid lipofuscinosis (cLINCL) is a monogenic disorder caused by the loss of tripeptidyl peptidase 1 (TPP1) activity as result mutations in CLN2. Absence TPP1 results lysosomal storage with an accompanying axonal degeneration throughout central nervous system (CNS), which leads to progressive neurodegeneration and early death. In this study, we compared efficacies pre- post-symptomatic injections recombinant adeno-associated virus (AAV) for treating cellular...
Niemann-Pick disease (NPD) is caused by the loss of acid sphingomyelinase (ASM) activity, which results in widespread accumulation undegraded lipids cells viscera and CNS. In this study, we tested effect combination brain systemic injections recombinant adeno-associated viral vectors encoding human ASM (hASM) a mouse model NPD. Animals treated therapy exhibited high levels hASM brain, resulted near-complete correction storage throughout body. This global reversal pathology translated to...
MicroRNAs (miRNAs) are a family of small non-protein coding RNAs, which regulate the expression wide variety genes at post-transcriptional level to control numerous biological and pathological processes. Various circulating miRNAs have been identified as potential diagnostic prognostic biomarkers in multiple types cancer disease. The aim present study was identify miRNA for early diagnosis relapse prediction osteosarcoma (OS). profiling performed on serum from patients with healthy controls....
Metachromatic leukodystrophy (MLD) is an autosomal recessive neurodegenerative disorder caused by mutations in the arylsulfatase A (ARSA) gene, resulting lower sulfatase activity and toxic accumulation of sulfatides central peripheral nervous system. Children account for 70% cases become progressively disabled with death occurring within 10 years disease onset. Gene therapy approaches to restore ARSA expression via adeno-associated viral vectors (AAV) have been promising but hampered limited...
The aim of this study was to screen for possible biomarkers metastatic osteosarcoma (OS) using a DNA microarray.We downloaded the gene expression profile GSE49003 from Gene Expression Omnibus database, which included 6 chips and non-metastatic OS patients. R package used identify differentially expressed genes (DEGs) between Then we compared DEGs in two groups sub-grouped into up-regulated down-regulated, followed by functional enrichment analysis DAVID system. Subsequently, constructed an...
Osteosarcoma is a primary cause of cancer-associated death in children and adolescents worldwide. Long non-coding RNAs SNHG16 (lncRNA SNHG16) integrin subunit-a 6 (ITGA6) are recently reported to be involved the tumorigenesis osteosarcoma by multiple mechanisms. However, correlation between ITGA6 remains undetermined. Expression miR-488, ITGA6, as well epithelial-mesenchymal transition (EMT) associated markers tissues cell lines were examined qRT-PCR or Western blotting. Effects on...