(Q30496159)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

title

Cystathionine gamma-Lyase-deficient mice require dietary cysteine to protect against acute lethal myopathy and oxidative injury (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

Makoto Suematsu

6

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

author name string

Noriyuki Akahoshi

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

Hidenori Yamada

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

Shintaro Nakano

4

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

Takashi Izumi

5

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

language of work or name

English

0 references

publication date

21 June 2010

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

Journal of Biological Chemistry

6 February 2020

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

volume

285

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

issue

34

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

page(s)

26358-26368

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

Muscle inactivation of mTOR causes metabolic and dystrophin defects leading to severe myopathy

6 February 2020

cites work

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

ATF4-dependent transcription mediates signaling of amino acid limitation

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Cystathionine gamma-lyase: Clinical, metabolic, genetic, and structural studies

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Mouse models of cystathionine beta-synthase deficiency reveal significant threshold effects of hyperhomocysteinemia

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

H2S as a physiologic vasorelaxant: hypertension in mice with deletion of cystathionine gamma-lyase

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Homeostatic levels of p62 control cytoplasmic inclusion body formation in autophagy-deficient mice

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Hydrogen sulphide and its therapeutic potential

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Folate and neural tube defects

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Introduction to the newborn screening fact sheets

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Methionine flux to transsulfuration is enhanced in the long living Ames dwarf mouse

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

The central nervous system in animal models of hyperhomocysteinemia

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Expression of mutant human cystathionine beta-synthase rescues neonatal lethality but not homocystinuria in a mouse model

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Age-associated oxidative damage leads to absence of gamma-cystathionase in over 50% of rat lenses: relevance in cataractogenesis

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Vanin-1-/- mice exhibit a glutathione-mediated tissue resistance to oxidative stress

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Sulfur amino acid metabolism: pathways for production and removal of homocysteine and cysteine

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Murine cystathionine gamma-lyase: complete cDNA and genomic sequences, promoter activity, tissue distribution and developmental expression

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Tandem mass spectrometry in newborn screening: a primer for neonatal and perinatal nurses

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

The atherogenic effect of excess methionine intake

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

An integrated stress response regulates amino acid metabolism and resistance to oxidative stress

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Genomic basis of cystathioninuria (MIM 219500) revealed by multiple mutations in cystathionine gamma-lyase (CTH).

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Hyperhomocysteinemia: a new risk factor for degenerative diseases.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Role of antioxidants in paraquat toxicity

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Selective loss of sphingosine 1-phosphate signaling with no obvious phenotypic abnormality in mice lacking its G protein-coupled receptor, LP(B3)/EDG-3

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Endothelial dysfunction and elevation of S-adenosylhomocysteine in cystathionine beta-synthase-deficient mice.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

The quantitatively important relationship between homocysteine metabolism and glutathione synthesis by the transsulfuration pathway and its regulation by redox changes

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Pathways and regulation of homocysteine metabolism in mammals

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Homocysteine and its disulfide derivatives: a suggested consensus terminology

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Reduction of false negative results in screening of newborns for homocystinuria

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Newborn screening for homocystinuria: Irish and world experience

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Alpha-mannosidase-II deficiency results in dyserythropoiesis and unveils an alternate pathway in oligosaccharide biosynthesis.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Biochemical and clinical study of muscle atrophy at thenar and hypothenar eminences in patients with cirrhosis.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

A novel negative selection for homologous recombinants using diphtheria toxin A fragment gene.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Mice deficient in cystathionine beta-synthase: animal models for mild and severe homocyst(e)inemia

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Effect of D,L-propargylglycine on cystathionine metabolism in rats.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Cystathioninuria in Two Healthy Siblings

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Absence of Cystathionase in Human Fetal Liver: Is Cystine Essential?

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Deficiencies of cystathionase and homoserine dehydratase activities in cystathioninuria

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Cystathionase deficiency in fibroblast cultures from a patient with primary cystathioninuria.

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Newer concepts of the indispensable amino acids

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Mitochondrial damage in muscle occurs after marked depletion of glutathione and is prevented by giving glutathione monoester

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Impairment of cysteine synthesis from methionine in rats exposed to surgical stress

14 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Cystathionine beta-synthase as a carbon monoxide-sensitive regulator of bile excretion

14 July 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

Hyperhomocysteinemia accelerates atherosclerosis in cystathionine beta-synthase and apolipoprotein E double knock-out mice with and without dietary perturbation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

Abnormal lipid metabolism in cystathionine beta-synthase-deficient mice, an animal model for hyperhomocysteinemia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

Genetic background conversion ameliorates semi-lethality and permits behavioral analyses in cystathionine beta-synthase-deficient mice, an animal model for hyperhomocysteinemia

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

Elevated plasma total homocysteine in severe methionine adenosyltransferase I/III deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

A Quantitative Assessment of Plasma Homocysteine as a Risk Factor for Vascular Disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

Autophagy is required to maintain muscle mass.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

Infantile hypermethioninemia and hyperhomocysteinemia due to high methionine intake: a diagnostic trap

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

Cellular redox state and endothelial dysfunction in mildly hyperhomocysteinemic cystathionine beta-synthase-deficient mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

Cystathionine beta-synthase is enriched in the brains of Down's patients.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

Inhibition of cystathionine-gamma-lyase leads to loss of glutathione and aggravation of mitochondrial dysfunction mediated by excitatory amino acid in the CNS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

Newborn population screening for classic homocystinuria by determination of total homocysteine from Guthrie cards

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M110.147439

New enzymatic colorimetric assay for total homocysteine

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2924062

Maternal gamma-cystathionase deficiency: absence of both teratogenic effects and pregnancy complications

26 November 2018

1 reference

12 December 2020

The effect of folic acid fortification on plasma folate and total homocysteine concentrations

1 reference

12 December 2020

1 reference

12 December 2020

Differential induction of cystathionine gamma-lyase in the livers and kidneys of mouse dams during gestation and lactation

1 reference

12 December 2020

A new HPLC method for the simultaneous determination of oxidized and reduced plasma aminothiols using coulometric electrochemical detection

1 reference

12 December 2020

Effects of age on levels of cysteine, glutathione and related enzyme activities in livers of mice and rats and an attempt to replenish hepatic glutathione level of mouse with cysteine derivatives

1 reference

12 December 2020

1 reference

12 December 2020

Liver intracellular L-cysteine concentration is maintained after inhibition of the trans-sulfuration pathway by propargylglycine in rats

1 reference

12 December 2020

The development of cystathionase activity during the first year of life

1 reference

12 December 2020

L-cysteine and glutathione metabolism are impaired in premature infants due to cystathionase deficiency

1 reference

12 December 2020

Identifiers

DOI

10.1074/JBC.M110.147439

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:20566639%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

1 reference