PathWhiz

PathWhiz ID	Pathway	Meta Data
PW002530 View Pathway	metabolic Cysteine Metabolism Arabidopsis thaliana BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: miguel ramirez Created On: April 18, 2016 at 17:16 Last Updated: April 18, 2016 at 17:16
PW002383 View Pathway	metabolic Cysteine Metabolism Saccharomyces cerevisiae The biosynthesis of cysteine begins with aspartate being phosphorylated into L-aspartyl-4-phosphate through an ATP driven aspartate kinase. L-aspartyl-4-phosphate is then catabolized through an NADPH dependent Aspartic Beta-Semiladehyde dehydrogenase resulting in the release of L-aspartate semialdehyde which is transformed into a homoserine through a Homoserine dehydrogenase. Homeserine in turn is acetylated through a homoserine O-trans-acetylase resulting in the release of O-acetyl-L-homoserine. The latter compound interacts with hydrogen sulfide through a O-acetylhomoserine (thiol)-lyase resulting in the release of L-homocysteine. L-homocysteine reacts with serine through a cystathionine beta synthase resulting in the release of water and L-cystathionine. This compound in turn can be turned into cysteine by reacting with water through a cystathionine gama-lyase. Cysteine can be turned back to L-cystathionine by reacting with a acetyl-L-homoserine spontaneously, thus resulting in L-cystathionine. Cysteine can also be degraded by reacting with a cystathionine gamma lyase resulting in the release of hydrogen sulfide, a hydrogen ion and 2-aminoprop-2-enoate which can spontaneously be converted into 2-iminopropanoate and further degraded into pyruvic acid. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: miguel ramirez Created On: December 10, 2015 at 15:09 Last Updated: December 10, 2015 at 15:09
PW064583 View Pathway	metabolic Cysteine Metabolism Mus musculus The semi-essential amino aid cysteine is tightly regulated in the body to ensure proper levels for metabolism but maintaining levels below toxic thresholds. Cysteine can be obtained from diet or synthesized from O-acetyl-L-serine. Cystine is the dimeric form of cysteine. Cysteine is a precursor for protein synthesis and an antioxidant. Impaired cysteine metabolism has been linked with neurodegenerative disorders. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Carin Li Created On: January 21, 2018 at 20:36 Last Updated: January 21, 2018 at 20:36
PW144280 View Pathway	drug action Cystine Drug Metabolism Action Pathway Homo sapiens BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: October 07, 2023 at 13:05 Last Updated: October 07, 2023 at 13:05
PW000699 View Pathway	disease Cystinosis, Ocular Nonnephropathic Homo sapiens Ocular non-nephropathic cystinosis, also known as adult-onset cystinosis, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of the cysteine metabolism pathway. It is caused by a defect in the CTNS gene, which encodes the protein cystinosin, which acts as a cystine/H+ symporter that transports L-cysteine out of the lysosome. Ocular non-nephropathic cystinosis is characterized by a buildup of cysteine in cells, in the case of this form in the cornea. Symptoms include photophobia and damage to the cornea due to crystals forming from the excess cysteine. However, unlike other forms of cystinosis, no or minimal kidney damage occurs. Treatment with cysteamine, a drug that can convert cysteine into a form that can be secreted by the lysosome, can be effective in all of the forms of cystinosis. It is estimated that ocular non-nephropathic cystinosis affects less than 1 in 100,000 to 200,000 individuals, which is the rate of the more severe nephropathic cystinosis. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: WishartLab Created On: June 23, 2014 at 02:48 Last Updated: June 23, 2014 at 02:48
PW121910 View Pathway	disease Cystinosis, Ocular Nonnephropathic Mus musculus Ocular non-nephropathic cystinosis, also known as adult-onset cystinosis, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of the cysteine metabolism pathway. It is caused by a defect in the CTNS gene, which encodes the protein cystinosin, which acts as a cystine/H+ symporter that transports L-cysteine out of the lysosome. Ocular non-nephropathic cystinosis is characterized by a buildup of cysteine in cells, in the case of this form in the cornea. Symptoms include photophobia and damage to the cornea due to crystals forming from the excess cysteine. However, unlike other forms of cystinosis, no or minimal kidney damage occurs. Treatment with cysteamine, a drug that can convert cysteine into a form that can be secreted by the lysosome, can be effective in all of the forms of cystinosis. It is estimated that ocular non-nephropathic cystinosis affects less than 1 in 100,000 to 200,000 individuals, which is the rate of the more severe nephropathic cystinosis. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: September 10, 2018 at 15:50 Last Updated: September 10, 2018 at 15:50
PW122134 View Pathway	disease Cystinosis, Ocular Nonnephropathic Rattus norvegicus Ocular non-nephropathic cystinosis, also known as adult-onset cystinosis, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of the cysteine metabolism pathway. It is caused by a defect in the CTNS gene, which encodes the protein cystinosin, which acts as a cystine/H+ symporter that transports L-cysteine out of the lysosome. Ocular non-nephropathic cystinosis is characterized by a buildup of cysteine in cells, in the case of this form in the cornea. Symptoms include photophobia and damage to the cornea due to crystals forming from the excess cysteine. However, unlike other forms of cystinosis, no or minimal kidney damage occurs. Treatment with cysteamine, a drug that can convert cysteine into a form that can be secreted by the lysosome, can be effective in all of the forms of cystinosis. It is estimated that ocular non-nephropathic cystinosis affects less than 1 in 100,000 to 200,000 individuals, which is the rate of the more severe nephropathic cystinosis. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: September 10, 2018 at 15:52 Last Updated: September 10, 2018 at 15:52
PW127177 View Pathway	disease Cystinosis, Ocular Nonnephropathic Homo sapiens Ocular non-nephropathic cystinosis, also known as adult-onset cystinosis, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of the cysteine metabolism pathway. It is caused by a defect in the CTNS gene, which encodes the protein cystinosin, which acts as a cystine/H+ symporter that transports L-cysteine out of the lysosome. Ocular non-nephropathic cystinosis is characterized by a buildup of cysteine in cells, in the case of this form in the cornea. Symptoms include photophobia and damage to the cornea due to crystals forming from the excess cysteine. However, unlike other forms of cystinosis, no or minimal kidney damage occurs. Treatment with cysteamine, a drug that can convert cysteine into a form that can be secreted by the lysosome, can be effective in all of the forms of cystinosis. It is estimated that ocular non-nephropathic cystinosis affects less than 1 in 100,000 to 200,000 individuals, which is the rate of the more severe nephropathic cystinosis. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: November 02, 2022 at 11:52 Last Updated: November 02, 2022 at 11:52
PW121911 View Pathway	disease Cystinuria Mus musculus Cystinuria is a genetic condition caused by mutations in the SLC7A9 or SLC3A1 gene. These two genes are responsible for creating subunits of a protein that reabsorbs cystine into the blood, located in the kidneys. The mutations cause this process to be compromised and allows the amino acids to build up and have a high concentration in urine. This causes crystals to form and become stones as they grow larger. These stones can become lodged in the bladder or in the kidneys which can cause pain, develop infection and disrupt the passing of urine through the urinary tract if the stones create a blockage there. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: September 10, 2018 at 15:50 Last Updated: September 10, 2018 at 15:50
PW122135 View Pathway	disease Cystinuria Rattus norvegicus Cystinuria is a genetic condition caused by mutations in the SLC7A9 or SLC3A1 gene. These two genes are responsible for creating subunits of a protein that reabsorbs cystine into the blood, located in the kidneys. The mutations cause this process to be compromised and allows the amino acids to build up and have a high concentration in urine. This causes crystals to form and become stones as they grow larger. These stones can become lodged in the bladder or in the kidneys which can cause pain, develop infection and disrupt the passing of urine through the urinary tract if the stones create a blockage there. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: September 10, 2018 at 15:52 Last Updated: September 10, 2018 at 15:52