PathWhiz

PathWhiz ID	Pathway	Meta Data
PW088522 View Pathway	metabolic Phosphatidylinositol Phosphate Metabolism Caenorhabditis elegans Phosphatidylinositol phosphates, or phosphoinositides, are intracellular signaling lipids. Seven different phosphoinositides have been identified in mammals, each distinguished by the number and/or position of the phosphate groups on the inositol ring. The inositol can be mono-, di-, or triphosphorylated, with the remaining phosphoinositides being isomers of these three forms. Phosphoinositides regulate a variety of signal transduction processes, thus playing a number of important roles in the cell, such as actin cytoskeletal reorganization, membrane transport, and cell proliferation. They may also affect protein localization, aggregation, and activity by acting as secondary messengers. The ability of the cell to recognize the different types of phosphoinositides as different cellular signals means that their synthesis and metabolism must be tightly regulated. Synthesis begins with the attachment of an inositol phosphate head group to diacylglycerol via a phospholipase C enzyme, creating a phosphoinositide. Conversion between the different types of phosphoinositides is then done by a number of specific phosphoinositide kinases and phosphatases, which add (kinase) and remove (phosphatase) phosphates from the inositol ring. The specific localization and regulation of the phosphoinositide kinases and phosphatases thus controls the activity of the phosphoinositides. While the phosphoinositides are always located in the membrane, their particular kinases and phosphatases may be found in the cytoplasm or in the membrane of the cell or cell organelles. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: August 10, 2018 at 18:10 Last Updated: August 10, 2018 at 18:10
PW122104 View Pathway	disease Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1) Rattus norvegicus Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1), also called Phosphoenolpyruvate carboxykinase-1 (PCK1) deficiency, Phosphopyruvate carboxylase deficiency, Phosphoenolpyruvate carboxylase deficiency, Phosphoenolpyruvate carboxykinase deficiency, or PEP carboxykinase deficiency, is a rare inborn error of metabolism (IEM) and an autosomal recessive disorder of gluconeogenesis caused by a deficient PEPCK1 enzyme. PEPCK1 catalyzes the conversion of amino acids into sugars, mainly glucose, which is important in preventing hypoglycemia. This disorder is characterized by a large accumulation of lactic acid in the blood. Symptoms of the disorder include hepatomegaly, failure to thrive and liver failure, depending on the severity of the case. Treatment including heavy carbohydrates and fasting is very effective. It is estimated that Phosphoenolpyruvate Carboxykinase Deficiency 1 has only affected 10 individuals around the world according to medical literature. 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
PW000536 View Pathway	disease Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1) Homo sapiens Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1), also called Phosphoenolpyruvate carboxykinase-1 (PCK1) deficiency, Phosphopyruvate carboxylase deficiency, Phosphoenolpyruvate carboxylase deficiency, Phosphoenolpyruvate carboxykinase deficiency, or PEP carboxykinase deficiency, is a rare inborn error of metabolism (IEM) and an autosomal recessive disorder of gluconeogenesis caused by a deficient PEPCK1 enzyme. PEPCK1 catalyzes the conversion of amino acids into sugars, mainly glucose, which is important in preventing hypoglycemia. This disorder is characterized by a large accumulation of lactic acid in the blood. Symptoms of the disorder include hepatomegaly, failure to thrive and liver failure, depending on the severity of the case. Treatment including heavy carbohydrates and fasting is very effective. It is estimated that Phosphoenolpyruvate Carboxykinase Deficiency 1 has only affected 10 individuals around the world according to medical literature. 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
PW121880 View Pathway	disease Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1) Mus musculus Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1), also called Phosphoenolpyruvate carboxykinase-1 (PCK1) deficiency, Phosphopyruvate carboxylase deficiency, Phosphoenolpyruvate carboxylase deficiency, Phosphoenolpyruvate carboxykinase deficiency, or PEP carboxykinase deficiency, is a rare inborn error of metabolism (IEM) and an autosomal recessive disorder of gluconeogenesis caused by a deficient PEPCK1 enzyme. PEPCK1 catalyzes the conversion of amino acids into sugars, mainly glucose, which is important in preventing hypoglycemia. This disorder is characterized by a large accumulation of lactic acid in the blood. Symptoms of the disorder include hepatomegaly, failure to thrive and liver failure, depending on the severity of the case. Treatment including heavy carbohydrates and fasting is very effective. It is estimated that Phosphoenolpyruvate Carboxykinase Deficiency 1 has only affected 10 individuals around the world according to medical literature. 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
PW127358 View Pathway	disease Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1) Homo sapiens Phosphoenolpyruvate Carboxykinase Deficiency 1 (PEPCK1), also called Phosphoenolpyruvate carboxykinase-1 (PCK1) deficiency, Phosphopyruvate carboxylase deficiency, Phosphoenolpyruvate carboxylase deficiency, Phosphoenolpyruvate carboxykinase deficiency, or PEP carboxykinase deficiency, is a rare inborn error of metabolism (IEM) and an autosomal recessive disorder of gluconeogenesis caused by a deficient PEPCK1 enzyme. PEPCK1 catalyzes the conversion of amino acids into sugars, mainly glucose, which is important in preventing hypoglycemia. This disorder is characterized by a large accumulation of lactic acid in the blood. Symptoms of the disorder include hepatomegaly, failure to thrive and liver failure, depending on the severity of the case. Treatment including heavy carbohydrates and fasting is very effective. It is estimated that Phosphoenolpyruvate Carboxykinase Deficiency 1 has only affected 10 individuals around the world according to medical literature. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: December 15, 2022 at 15:17 Last Updated: December 15, 2022 at 15:17
PW064768 View Pathway	signaling Phospholipase C Signaling Pathway Homo sapiens Calcium signaling is a process whereby the extremely low cytoplasmic Ca2+ concentration increases in a deliberate and specific manner to trigger downstream cellular events. Phospholipase C can produce (or modulate), directly, three distinct signals: inositol 1,4,5-trisphosphate (IP3), diacylglycerol, and phosphatidylinositol 4,5-bisphosphate (PIP2). BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Noah Created On: June 18, 2018 at 10:21 Last Updated: June 18, 2018 at 10:21
PW101508 View Pathway	signaling Phospholipase C Signaling Pathway Bos taurus Phospholipase C pathways is one of the major intracellular signalling pathways regulating hormones. It functions to activate inositol lipid signalling pathways causing the hydrolysis of PIP2 by PLC to IP3 and diacylglycerol to activate protein kinase C. IP3 releases calcium from the endoplasmic reticulum. PIP3 is an important regulator of AKT signaling and downstream pathways. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: August 23, 2018 at 12:22 Last Updated: August 23, 2018 at 12:22
PW101523 View Pathway	signaling Phospholipase C Signaling Pathway Rattus norvegicus Phospholipase C pathways is one of the major intracellular signalling pathways regulating hormones. It functions to activate inositol lipid signalling pathways causing the hydrolysis of PIP2 by PLC to IP3 and diacylglycerol to activate protein kinase C. IP3 releases calcium from the endoplasmic reticulum. PIP3 is an important regulator of AKT signaling and downstream pathways. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: August 23, 2018 at 12:26 Last Updated: August 23, 2018 at 12:26
PW101493 View Pathway	signaling Phospholipase C Signaling Pathway Mus musculus Phospholipase C pathways is one of the major intracellular signalling pathways regulating hormones. It functions to activate inositol lipid signalling pathways causing the hydrolysis of PIP2 by PLC to IP3 and diacylglycerol to activate protein kinase C. IP3 releases calcium from the endoplasmic reticulum. PIP3 is an important regulator of AKT signaling and downstream pathways. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: August 23, 2018 at 12:19 Last Updated: August 23, 2018 at 12:19
PW064775 View Pathway	protein Phospholipase C Signaling Pathway Homo sapiens Phospholipase C pathways is one of the major intracellular signalling pathways regulating hormones. It functions to activate inositol lipid signalling pathways causing the hydrolysis of PIP2 by PLC to IP3 and diacylglycerol to activate protein kinase C. IP3 releases calcium from the endoplasmic reticulum. PIP3 is an important regulator of AKT signaling and downstream pathways. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Debra Lipton Created On: June 21, 2018 at 13:35 Last Updated: June 21, 2018 at 13:35