Browse
Submit Data
Databases
API
Help

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Novel mechanisms of galactose toxicity in human galactose-1-phosphate uridyltransferase (GALT)-deficient cells

ABSTRACT: Comparison of gene expression profiles in GALT-deficient and GALT-reconstituted cells showed that cells lacking GALT activity responded to galactose challenge by activating a set of genes characteristic of endoplasmic reticulum (ER) stress. This response was specific to galactose insult, as cells grown in glucose or hexose-free media did not exhibit ER stress. Keywords: GALT infected cells

ORGANISM(S): Homo sapiens

PROVIDER: GSE4864 | GEO | 2007/11/01

SECONDARY ACCESSION(S): PRJNA95751

REPOSITORIES: GEO

ACCESS DATA

Json Xml

Dataset's files

Source:

			Action	DRS
		Other

Items per page:

1 - 1 of 1

Similar Datasets

Transcription profiling of human galactose-1-phosphate uridyltransferase (GALT)-deficient cells reveals novel mechanisms of galactose toxicity

Project description:Comparison of gene expression profiles in GALT-deficient and GALT-reconstituted cells showed that cells lacking GALT activity responded to galactose challenge by activating a set of genes characteristic of endoplasmic reticulum (ER) stress. This response was specific to galactose insult, as cells grown in glucose or hexose-free media did not exhibit ER stress. Experiment Overall Design: Cells were first grown and then infected in high glucose, then transferred to DMEM, 3 experiments with dye-swap

2008-06-13 | E-GEOD-4864 | biostudies-arrayexpress

Transcription profiling of wild type, GALT-deficient and GALT-deficient-revertant yeast grown on media with galactose or glucose

Project description:To better understand the pathophysiology of galactose-1-phosphate uridyltransferase (GALT) deficiency in humans, we studied the mechanisms by which a GALT-deficient yeast survived on galactose medium. Under normal conditions, GALT-deficient yeast cannot grow in medium that contains 0.2% galactose as the sole carbohydrate, a phenotype of Gal(-). We isolated revertants from a GALT-deficient yeast by direct selection for growth in galactose, a phenotype of Gal(+). Comparison of gene expression profiles among wild-type and revertant strains on galactose medium revealed that the revertant down-regulated genes encoding enzymes including galactokinase, galactose permease, and UDP-galactose-4-epimerase (the GAL regulon). By contrast, the revertant strain up-regulated the gene for UDP-glucose pyrophosphorylase, UGP1. There was reduced accumulation of galactose-1-phosphate in the galactose-grown revertant cells when compared to the GALT-deficient parent cells.

2008-04-10 | E-MEXP-1420 | biostudies-arrayexpress

Tiling microarray analysis of gene expression profile of Escherichia coli galT mutant cells under D-galactose-stressed condition

Project description:To understand the altered gene expression by D-galactose in galT cells, the cells were grown in the presence and absence of D-galactose and tiling array analysis was performed for transcriptome profiling. A galT defect causes the disease Galactosemia in human. This work helps us to study the human metabolic disease Galactosemia. Total RNAs were isolated from Escherichia coli galT strain grown in the absence and presence of D-galactose. The transcriptome profile of galT cells was figured out in the galactose-stressed condition.

2013-12-17 | E-GEOD-42821 | biostudies-arrayexpress

Tiling microarray analysis of gene expression profile of Escherichia coli galT mutant cells under D-galactose-stressed condition

2013-12-17 | GSE42821 | GEO

Transcription profiling of mouse GalT knockout recipient of GalT transducded allo-bone marrow cells after sublethal irradiation

Project description:Xenotransplantation holds the promise of providing an unlimited supply of donor organs for terminal patients with organ failure. The gal carbohydrate results in rejection of wild type pig grafts, however, chimerism established by expression of the GalT gene prior to transplantation in GalT knockout mice results in tolerance to Gal+ heart grafts. We used microarrays in order to further understand the early events that occur within grafts that demonstrate tolerance. Experiment Overall Design: The GalT BMT recipient is a GalT knockout mouse which recieved GalT gene transduced allo-bone marrow cells transplantation after sublethal irradiation. A heart of wild type C57BL/6 was heterotopically transplanted into the recipient after GalT BMT. Syngeneic Control recipient is a wild type C57BL/6 transplanted a heart of wild C57BL/6.

2008-06-15 | E-GEOD-7424 | biostudies-arrayexpress

A comparison between GalT-/-;hCD39;hCD55 and GalT-/-;hCD39;hCD46;hCD55;TBM pig kidney transplanted in non-human primates

Project description:Due to shortage of donor kidney, genetically modified kidney xenograft from pigs is considered. To select the optimal gene modification for xenotransplantation, knockout of carbohydrate genes or knockin of protective proteins have been applied. In this study, we utilized GalT- /-;hCD39;hCD55 and GalT-/-;hCD39;hCD46;hCD55;thrombomodulin (TBM) pigs for transplantation in non-human primates. NHPs survived for 4 weeks after kidney transplantation (4 WAT) from GalT-/-;hCD39;hCD55 pig and 6 WAT from GalT- /-;hCD39;hCD46;hCD55;TBM pig. However, mRNA sequencing and immunohistochemistry analysis revealed that 6 WAT kidney showed the severe apoptosis, inflammation, loss of renal function, and renal fibrosis than 4 WAT kidney. These results suggest that additional knockin of anti-complement (hCD26) and anti-coagulation (TBM) is not sufficient to inhibit the renal damage.

2023-12-31 | GSE240012 | GEO

Single-Cell RNA Sequencing of Galt Gene-Edited Mouse Lung Tissue

Project description:This dataset contains single-cell RNA sequencing data from lung tissues of Galt gene-edited (GAL) mice and wild-type (WT) mice. The samples were collected for the investigation of molecular changes induced by Galt gene mutations, specifically focusing on lung injury. The dataset includes gene expression profiles of individual cells and enables the study of cell-type-specific responses, immune cell changes, and alterations in key cellular pathways. This dataset is useful for further understanding the pathophysiology of lung injury associated with Galt gene mutations and provides a resource for research into the molecular mechanisms of galactosemia.

2025-01-01 | GSE283551 | GEO

Transcriptional profiling of Myceliophthora thermophila on galactose and metabolic engineering for improved galactose utilization

Project description:Transcriptional landscape of MyceliopTranscriptional profiling of Myceliophthora thermophila on galactose and metabolic engineering for improved galactose utilization thora thermophila responding to soluble starch and the role of regulator AmyR on polysaccharide degradation Efficient biological conversion of all sugars from lignocellulosic biomass is necessary for cost-effective production of biofuels and commodity chemicals. Galactose is the next most abundant sugar in many hemicelluloses and it will be important to capture this carbon for an efficient bioconversion process of plant biomass. Thermophilic fugus Myceliophthora thermophila has been used as cell factory to produce biochemicals directly from renewable polysaccharides. This study determined the transcriptomic profiles of M. thermophila responding to galactose and identified the gene involved in the oxido-reductive pathway for galactose degradation, of which hexokinase might be the rate-limiting enzyme in this thermophilic fungus. Galactokinase was necessary for high induction of galactose transporter and disruption of galK resulted in decreased galactose utilization. Both of galactokinase deletion and sugar transporter overexpression could further activate the oxido-reductive pathway and led to improved galactose utilization. In addition, disruption of the Leloir pathway brought about reduced tolerance to cell wall perturbation, oxidative stress and high osmolality. In order to accelerate galactose consumption, the third galactose-degradation pathway- De Ley-Doudoroff pathway was successfully integrated into M. thermophila and the consumption rate of galactose was increased by 57%. This study will be benefit for the rational design of fungal strains to produce biofuels and biochemical from plant biomass, especially, marine plant biomass with the abundant of galactose, such as red seaweed and molasses.

2021-02-05 | GSE165516 | GEO

Early Postnatal Alterations in Follicular Stress Response and Survival in a Mouse Model of Classic Galactosemia

Project description:Primary ovarian insufficiency (POI) is characterized by accelerated loss of primordial follicles, which results in ovarian failure and concomitant menopause before age 40. 1-3% of females in the general population are diagnosed with POI and 80% of females with the inherited disease Classic Galactosemia (CG) will develop POI. CG is caused by mutations in the GALT gene encoding the enzyme galactose-1-phosphate uridylyltransferase. While dietary restriction of galactose is lifesaving in the neonatal period, the development of severe complications including POI is not mitigated. Additionally, the pattern of follicle loss have not been completely characterized. The chronic accumulation of aberrant metabolites such as galactose-1 phosphate and galactitol are the suspected culprits in the development of the sequelae, yet the mechanisms remain elusive. Our group uses a GalT gene-trapped mouse model to study the pathophysiology of CG. Recently, we showed that alterations in the Integrated Stress Response pathway occur in the ovaries of these mice, likely contributing to the POI phenotype. Using immunofluorescent staining in whole-ovary histology at progressive ages, we saw evidence of altered Integrated Stress Response activity in granulosa cells and primordial oocytes leading to accelerated primordial follicle growth, aberrant DNA damage repair, and increased cellular stress/death. RNA-sequencing of whole ovary revealed significant alterations in cholesterol/lipid metabolism and transcriptional regulation. Overall, our findings indicate abnormal stress response results in accelerated primordial follicle activation or “burnout,” which may explain the POI phenotype of fewer primordial follicles at later ages.

2023-02-01 | GSE196454 | GEO

Impact of oxidative stress on ascorbate biosynthesis in Chlamydomonas via regulation of the VTC2 gene encoding a GDP-l-galactose phosphorylase

Project description:The l-galactose (Smirnoff-Wheeler) pathway represents the major route to l-ascorbic acid (vitamin C) biosynthesis in plants. Arabidopsis thaliana VTC2 and its paralogue VTC5 function as GDP-l-galactose phosphorylases converting GDP-l-galactose to l-galactose-1-P, thus catalyzing the first committed step in the biosynthesis of l-ascorbate. Here we report that the l-galactose pathway of ascorbate biosynthesis described in higher plants is conserved in green algae. The Chlamydomonas reinhardtii genome encodes all the enzymes required for vitamin C biosynthesis via the Smirnoff-Wheeler pathway. We have characterized recombinant C. reinhardtii VTC2 as an active GDP-l-galactose phosphorylase. C. reinhardtii cells exposed to oxidative stress show increased VTC2 mRNA and l-ascorbate levels. We have also shown that enzymatic components of the ascorbate-glutathione system (e.g. ascorbate peroxidase, Mn superoxide dismutase, dehydroascorbate reductase) are up-regulated in response to increased oxidative stress. These results indicate that C. reinhardtii VTC2, like its plant homologs, is a key enzyme in ascorbate biosynthesis in green algae and together with components of the ascorbate recycling system represents the major route in providing protective levels of ascorbate in oxidatively stressed algal cells. Our results suggest that C. reinhardtii cells exposed to oxidative stress conditions produce more ascorbate both by de novo synthesis (Smirnoff-Wheeler pathway) and by recycling via the ascorbate-glutathione cycle.

2012-05-03 | GSE34826 | GEO

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

Tweets

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data