PathWhiz

PathWhiz ID	Pathway	Meta Data
PW145902 View Pathway	drug action Isoaminile Drug Metabolism Action Pathway Homo sapiens BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: October 07, 2023 at 16:56 Last Updated: October 07, 2023 at 16:56
PW176075 View Pathway	metabolic Isoaminile Predicted Metabolism Pathway new Homo sapiens Metabolites of Isoaminile are predicted with biotransformer. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Omolola Created On: November 29, 2023 at 13:45 Last Updated: November 29, 2023 at 13:45
PW127240 View Pathway	disease Isobutyryl-CoA Dehydrogenase Deficiency Homo sapiens Isobutyryl-CoA dehydrogenase deficiency, also called IBDD, is an extremely rare inherited inborn error of metabolism (IEM) of valine metabolism. It is an autosomal recessive disorder that is caused by a defective isobutyryl-coenzyme A dehydrogenase. Approximately 30 people have been identified with this condition, although the frequency may be much higher since it is relatively asymptomatic. Isobutyryl-coenzyme A dehydrogenase is a mitochondrial protein that belongs to the acyl-CoA dehydrogenase family of enzymes. Its main function is to catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of branched-chain amino acids, specifically valine. This enzyme is responsible for the third step in the breakdown of valine and converts isobutyryl-CoA into methylacrylyl-CoA. Defects in the IBD enzyme function lead to elevated levels of valine in blood and other biofluids (valinemia). IBDD can be identified by elevated levels of C4-acylcarnitine via newborn screening. Most people with IBDD are asymptomatic. Some individuals with IBDD have developed features such as a weakened and enlarged heart (dilated cardiomyopathy), weak muscle tone (hypotonia), and developmental delay. This condition may also result in low numbers of red blood cells (anemia) and very low levels of carnitine in the blood, which is a compound that plays a role in converting certain foods into energy. Symptoms may be worsened by long periods of fasting or infections that increase the body's demand for energy. Treatment may include the use of L-carnitine supplements, frequent meals, and a low-valine diet. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: November 18, 2022 at 16:39 Last Updated: November 18, 2022 at 16:39
PW122068 View Pathway	disease Isobutyryl-CoA Dehydrogenase Deficiency Rattus norvegicus Isobutyryl-CoA dehydrogenase deficiency, also called IBDD, is an extremely rare inherited inborn error of metabolism (IEM) of valine metabolism. It is an autosomal recessive disorder that is caused by a defective isobutyryl-coenzyme A dehydrogenase. Approximately 30 people have been identified with this condition, although the frequency may be much higher since it is relatively asymptomatic. Isobutyryl-coenzyme A dehydrogenase is a mitochondrial protein that belongs to the acyl-CoA dehydrogenase family of enzymes. Its main function is to catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of branched-chain amino acids, specifically valine. This enzyme is responsible for the third step in the breakdown of valine and converts isobutyryl-CoA into methylacrylyl-CoA. Defects in the IBD enzyme function lead to elevated levels of valine in blood and other biofluids (valinemia). IBDD can be identified by elevated levels of C4-acylcarnitine via newborn screening. Most people with IBDD are asymptomatic. Some individuals with IBDD have developed features such as a weakened and enlarged heart (dilated cardiomyopathy), weak muscle tone (hypotonia), and developmental delay. This condition may also result in low numbers of red blood cells (anemia) and very low levels of carnitine in the blood, which is a compound that plays a role in converting certain foods into energy. Symptoms may be worsened by long periods of fasting or infections that increase the body's demand for energy. Treatment may include the use of L-carnitine supplements, frequent meals, and a low-valine diet. 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
PW121844 View Pathway	disease Isobutyryl-CoA Dehydrogenase Deficiency Mus musculus Isobutyryl-CoA dehydrogenase deficiency, also called IBDD, is an extremely rare inherited inborn error of metabolism (IEM) of valine metabolism. It is an autosomal recessive disorder that is caused by a defective isobutyryl-coenzyme A dehydrogenase. Approximately 30 people have been identified with this condition, although the frequency may be much higher since it is relatively asymptomatic. Isobutyryl-coenzyme A dehydrogenase is a mitochondrial protein that belongs to the acyl-CoA dehydrogenase family of enzymes. Its main function is to catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of branched-chain amino acids, specifically valine. This enzyme is responsible for the third step in the breakdown of valine and converts isobutyryl-CoA into methylacrylyl-CoA. Defects in the IBD enzyme function lead to elevated levels of valine in blood and other biofluids (valinemia). IBDD can be identified by elevated levels of C4-acylcarnitine via newborn screening. Most people with IBDD are asymptomatic. Some individuals with IBDD have developed features such as a weakened and enlarged heart (dilated cardiomyopathy), weak muscle tone (hypotonia), and developmental delay. This condition may also result in low numbers of red blood cells (anemia) and very low levels of carnitine in the blood, which is a compound that plays a role in converting certain foods into energy. Symptoms may be worsened by long periods of fasting or infections that increase the body's demand for energy. Treatment may include the use of L-carnitine supplements, frequent meals, and a low-valine diet. 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
PW000499 View Pathway	disease Isobutyryl-CoA Dehydrogenase Deficiency Homo sapiens Isobutyryl-CoA dehydrogenase deficiency, also called IBDD, is an extremely rare inherited inborn error of metabolism (IEM) of valine metabolism. It is an autosomal recessive disorder that is caused by a defective isobutyryl-coenzyme A dehydrogenase. Approximately 30 people have been identified with this condition, although the frequency may be much higher since it is relatively asymptomatic. Isobutyryl-coenzyme A dehydrogenase is a mitochondrial protein that belongs to the acyl-CoA dehydrogenase family of enzymes. Its main function is to catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of branched-chain amino acids, specifically valine. This enzyme is responsible for the third step in the breakdown of valine and converts isobutyryl-CoA into methylacrylyl-CoA. Defects in the IBD enzyme function lead to elevated levels of valine in blood and other biofluids (valinemia). IBDD can be identified by elevated levels of C4-acylcarnitine via newborn screening. Most people with IBDD are asymptomatic. Some individuals with IBDD have developed features such as a weakened and enlarged heart (dilated cardiomyopathy), weak muscle tone (hypotonia), and developmental delay. This condition may also result in low numbers of red blood cells (anemia) and very low levels of carnitine in the blood, which is a compound that plays a role in converting certain foods into energy. Symptoms may be worsened by long periods of fasting or infections that increase the body's demand for energy. Treatment may include the use of L-carnitine supplements, frequent meals, and a low-valine diet. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: WishartLab Created On: August 29, 2013 at 10:39 Last Updated: August 29, 2013 at 10:39
PW128296 View Pathway	drug action Isocarboxazid Amine Oxidase Norepinephrine Antidepressant Action Pathway Homo sapiens Isocarboxazid, also known as Marplan, is an antidepressant from the monoamine oxidase inhibitors (MAOIs) drug class. This drug is indicated in different disorders like major depression, dysthymic disorder, atypical disorder, panic disorder, and phobic disorders. Isocarboxazid inhibits the MAO in the brain, resulting in the relief of depressive symptoms. The monoamine oxidase is an enzyme that catalyzes the oxidative deamination of many amines like serotonin, norepinephrine, epinephrine, and dopamine. There are 2 isoforms of this protein: A and B. The first one is found in cells located in the periphery and breakdown serotonin, norepinephrine, epinephrine, dopamine, and tyramine. The second one, the B isoform, breakdowns phenylethylamine, norepinephrine, epinephrine, dopamine, and tyramine. This isoform is found in the extracellular tissues and mostly in the brain. The mechanism of action of the MAOIs is still not determined, it is thought that they act by increasing free serotonin and norepinephrine concentrations and/or by altering the concentrations of other amines in the CNS. MAO A inhibition is thought to be more relevant to antidepressant activity than the inhibition caused by MAO B. Selective MAO B inhibitors have no antidepressant effects. This drug is administered as an oral tablet. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Daphnee Created On: August 16, 2023 at 12:36 Last Updated: August 16, 2023 at 12:36
PW128366 View Pathway	drug action Isocarboxazid Amine Oxidase Serotonin Antidepressant Action Pathway Homo sapiens Isocarboxazid, also known as Marplan, is an antidepressant from the monoamine oxidase inhibitors (MAOIs) drug class. This drug is indicated in different disorders like major depression, dysthymic disorder, atypical disorder, panic disorder, and phobic disorders. Isocarboxazid inhibits the MAO in the brain, resulting in the relief of depressive symptoms. The monoamine oxidase is an enzyme that catalyzes the oxidative deamination of many amines like serotonin, norepinephrine, epinephrine, and dopamine. There are 2 isoforms of this protein: A and B. The first one is found in cells located in the periphery and breakdown serotonin, norepinephrine, epinephrine, dopamine, and tyramine. The second one, the B isoform, breakdowns phenylethylamine, norepinephrine, epinephrine, dopamine, and tyramine. This isoform is found in the extracellular tissues and mostly in the brain. The mechanism of action of the MAOIs is still not determined, it is thought that they act by increasing free serotonin and norepinephrine concentrations and/or by altering the concentrations of other amines in the CNS. MAO A inhibition is thought to be more relevant to antidepressant activity than the inhibition caused by MAO B. Selective MAO B inhibitors have no antidepressant effects. This drug is administered as an oral tablet. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Daphnee Created On: August 24, 2023 at 12:31 Last Updated: August 24, 2023 at 12:31
PW145333 View Pathway	drug action Isocarboxazid Drug Metabolism Action Pathway Homo sapiens BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: October 07, 2023 at 15:35 Last Updated: October 07, 2023 at 15:35
PW127387 View Pathway	drug action Isoconazole Action Pathway Homo sapiens Isoconazole is an imidazole antifungal used for superficial skin and vaginal infections. Isoconazole, like other imidazole antifungals, inhibits lanosterol 14-alpha demethylase in the endoplasmic reticulum of fungal cells. Lanosterol 14-alpha demethylase is the enzyme that catalyzes the synthesis of 4,4'-dimethyl cholesta-8,14,24-triene-3-beta-ol from lanosterol. With this enzyme inhibited ergosterol synthesis cannot occur which causes a significant low concentration of ergosterol in the fungal cell. Ergosterol is essential in maintaining membrane integrity in fungi. Without ergosterol, the fungus cell cannot synthesize membranes thereby increasing fluidity and preventing growth of new cells. This leads to cell lysis which causes it to collapse and die. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: January 02, 2023 at 13:36 Last Updated: January 02, 2023 at 13:36