ethylmaleimide has been researched along with Cystinosis in 9 studies
Ethylmaleimide: A sulfhydryl reagent that is widely used in experimental biochemical studies.
Cystinosis: A metabolic disease characterized by the defective transport of CYSTINE across the lysosomal membrane due to mutation of a membrane protein cystinosin. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. In the KIDNEY, nephropathic cystinosis is a common cause of RENAL FANCONI SYNDROME.
| Excerpt | Relevance | Reference |
|---|---|---|
| "The activity of a cystine transport system in lysosomes prepared from the leukocytes of patients with cystinosis was found to be deficient." | 7.66 | Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis. ( Bashan, N; Bernardini, I; Gahl, WA; Schulman, JD; Tietze, F, 1982) |
| "In patients with cystinosis, the concentration of free cystine in leukocytes was 80 times greater than normal, and six times the normal content for their parents." | 7.64 | Increased cystine in leukocytes from individuals homozygous and heterozygous for cystinosis. ( Bradley, K; Schneider, JA; Seegmiller, JE, 1967) |
| " The detection limit for the determination of cystine is adequate to measure cystine in leukocytes and cultured fibroblasts for diagnosis of cystinosis and monitoring treatment with cysteamine." | 3.70 | New method for determining cystine in leukocytes and fibroblasts. ( Blom, H; de Graaf-Hess, A; Trijbels, F, 1999) |
| "The activity of a cystine transport system in lysosomes prepared from the leukocytes of patients with cystinosis was found to be deficient." | 3.66 | Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis. ( Bashan, N; Bernardini, I; Gahl, WA; Schulman, JD; Tietze, F, 1982) |
| "In patients with cystinosis, the concentration of free cystine in leukocytes was 80 times greater than normal, and six times the normal content for their parents." | 3.64 | Increased cystine in leukocytes from individuals homozygous and heterozygous for cystinosis. ( Bradley, K; Schneider, JA; Seegmiller, JE, 1967) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 7 (77.78) | 18.7374 |
| 1990's | 2 (22.22) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 0 (0.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Schneider, JA | 3 |
| Bradley, K | 1 |
| Seegmiller, JE | 1 |
| Gahl, WA | 3 |
| Tietze, F | 3 |
| Bashan, N | 2 |
| Steinherz, R | 1 |
| Schulman, JD | 3 |
| Bernardini, I | 1 |
| de Graaf-Hess, A | 1 |
| Trijbels, F | 1 |
| Blom, H | 1 |
| Hsiung, M | 1 |
| Yeo, YY | 1 |
| Itiaba, K | 1 |
| Crawhall, JC | 2 |
| Bowie, L | 1 |
| Gochman, N | 1 |
| Johnson, K | 1 |
| Coor, C | 1 |
| Salmon, RF | 1 |
| Quigley, R | 1 |
| Marver, D | 1 |
| Baum, M | 1 |
| Butler, JD | 1 |
| Oshima, RG | 1 |
| Willis, RC | 1 |
| Furlong, CE | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Renal Transplantation in Recipients With Nephropathic Cystinosis[NCT00074516] | 11 participants (Actual) | Observational | 2003-12-10 | Completed | |||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
9 other studies available for ethylmaleimide and Cystinosis
| Article | Year |
|---|---|
Increased cystine in leukocytes from individuals homozygous and heterozygous for cystinosis.
Topics: Acid Phosphatase; Cell Nucleus; Cysteine; Cystine; Cystinosis; Cytoplasmic Granules; Ethylmaleimide; | 1967 |
Defective cystine exodus from isolated lysosome-rich fractions of cystinotic leucocytes.
Topics: Cysteine; Cystine; Cystinosis; Ethylmaleimide; Half-Life; Heterozygote; Humans; Leukocytes; Lysosome | 1982 |
Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis.
Topics: Biological Transport; Carrier Proteins; Cysteine; Cystine; Cystinosis; Ethylmaleimide; Humans; Kinet | 1982 |
New method for determining cystine in leukocytes and fibroblasts.
Topics: Adult; Borohydrides; Bridged Bicyclo Compounds; Cell Extracts; Chromatography, High Pressure Liquid; | 1999 |
Cysteamine, penicillamine, glutathione, and their derivatives analyzed by automated ion exchange column chromatography.
Topics: Animals; Autoanalysis; Chromatography, Ion Exchange; Cysteamine; Cystinosis; Ethylmaleimide; Fibrobl | 1978 |
Automated microanalysis of sulfur containing amino acids and their derivatives.
Topics: Amino Acids; Autoanalysis; Cells, Cultured; Cysteine; Cystinosis; Disulfides; Ethylmaleimide; Fibrob | 1976 |
Role of adenosine triphosphate (ATP) and NaK ATPase in the inhibition of proximal tubule transport with intracellular cystine loading.
Topics: Adenosine Triphosphate; Animals; Biological Transport; Cystine; Cystinosis; Ethylmaleimide; Fanconi | 1991 |
Cysteamine depletes cystinotic leucocyte granular fractions of cystine by the mechanism of disulphide interchange.
Topics: Cystamine; Cysteamine; Cystine; Cystinosis; Cytoplasmic Granules; Disulfides; Ethylmaleimide; Half-L | 1985 |
Binding assays for amino acids. The utilization of a cystine binding protein from Escherichia coli for the determination of acid-soluble cystine in small physiological samples.
Topics: Amino Acids; Amino Acids, Diamino; Bacterial Proteins; Binding Sites; Binding, Competitive; Carbon R | 1974 |