The influence of substrate chirality was studied in the aqueous phase reforming (APR) over Pt/Al 2 O 3 in a continuous fixed-bed reactor at 225 °C. Two epimeric sugar alcohols, namely sorbitol and galactitol, were used for performance comparison. (abo.fi)
URINE4
RBC gal-1-p and urine galactitol were monitored during the follow-up visits in every child with DG galactosemia. (nih.gov)
Gal-1-p, urine galactitol, liver function tests, and FSH were tested at the time of study visit. (nih.gov)
The groups had statistically significant differences on RBC gal-1-p and urine galactitol at the 2 week, 1 month, 6 month, and 1 year time points. (nih.gov)
There was no statistical difference of gal-1-p or urine galactitol in group-I and -II at the time of study. (nih.gov)
Dulcitol1
Galactitol (dulcitol) is a sugar alcohol, the reduction product of galactose. (wikipedia.org)
Galactose-1-phospha2
The other common galactose metabolism defect is a defect in galactose-1-phosphate uridylyltransferase, an autosomal recessive disorder, which also causes a buildup of galactitol as a result of increased concentrations of galactose-1-phosphate and galactose. (wikipedia.org)
In the absence of GALT, galactose-1-phosphate accumulates, along with excessive galactose and its oxidative and reductive products galactitol and galactonate (not shown). (medscape.com)
Cataracts1
This disorder leads to cataracts caused by galactitol buildup. (wikipedia.org)
Escherichia1
Sequence of the gat operon for galactitol utilization from a wild-type strain EC3132 of Escherichia coli. (rhea-db.org)
Accumulation1
Experimental galactose neuropathy (EGN) was chosen since in this model, edema is due to the accumulation of galactitol, which does not directly damage nerve fibers, so that it was possible to study the role of endoneurial edema alone. (elsevierpure.com)
Reaction1
Galactitol is produced from galactose in a reaction catalyzed by aldose reductase. (wikipedia.org)
Time1
For the very first time galactitol was used in the APR process. (abo.fi)
Plasma galactitol1
Govorestat also significantly reduced plasma galactitol levels in both adults and children with Galactosemia. (gcs-web.com)
Galactosemia4
Urinary excretion of galactitol was determined in 95 normals (N/N), 67 galactosemic (G/G), and 39 compound heterozygotes for the Duarte and galactosemia genotype (D/G). Galactitol excretion is age-dependent in both normal individuals and patients with classic galactosemia on lactose-restricted diets. (nih.gov)
Galactosemia is a devastating disease that progressively worsens with age, despite adherence to a galactose-restricted diet, due to endogenous production of galactose by the body and subsequent conversion to the toxic metabolite, galactitol. (gcs-web.com)
Galactitol is a toxic metabolite responsible for tissue damage and long-term complications in Galactosemia. (gcs-web.com)
Govorestat (AT-007) is a potent, selective, CNS-penetrant aldose reductase (aldehyde reductase) inhibitor with IC50 of 0.1 nM, reduces toxic galactitol levels and prevents galactosemia disease complications in vivo. (probechem.com)
Galactose5
Galactitol is produced from galactose in a reaction catalyzed by aldose reductase. (wikipedia.org)
The other common galactose metabolism defect is a defect in galactose-1-phosphate uridylyltransferase, an autosomal recessive disorder, which also causes a buildup of galactitol as a result of increased concentrations of galactose-1-phosphate and galactose. (wikipedia.org)
African-American galactosemic patients with the S135L mutation of the galactose-1-phosphate uridyltransferase (GALT) gene also excreted abnormal quantities of galactitol. (nih.gov)
As a result, galactose and a related sugar called galactitol can build up, particularly in cells that make up the lens of the eye. (medlineplus.gov)
When not metabolized properly, galactose is converted to the toxic metabolite, galactitol, which causes neurological complications, including deficiencies in speech, cognition, behavior, and motor skills, and also results in juvenile cataracts and ovarian insufficiency (in women). (gcs-web.com)
Galactosemic1
In galactosemic patients who are homozygous for the Q188R mutation, urinary galactitol levels were fivefold to 10-fold higher than those of normal subjects of comparable age. (nih.gov)
Urine1
Only minor changes in her urine galactitol values were noted during the study period but the values stayed in the range of treated galactosaemia patients. (nih.gov)
Galactosemias1
Se puede formar en exceso en el cristalino en las GALACTOSEMIAS, una deficiencia de GALACTOCINASA. (bvsalud.org)
Data1
The Company believes that the clinical efficacy demonstrated to date, combined with galactitol biomarker data and a favorable safety profile, may support an NDA submission, and is seeking feedback from the FDA and alignment on the details of the submission. (gcs-web.com)
Levels1
In contrast to the decrease in urinary galactitol with age, plasma levels remain in a narrow concentration range with no significant difference with age. (nih.gov)