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PathWhiz ID Pathway Meta Data

PW121877

Pw121877 View Pathway
disease

Sucrase-Isomaltase Deficiency

Mus musculus
Congenital sucrase-isomaltase deficiency is a rare inborn error of metabolism (IEM) and autosomal recessive disorder caused by mutatins in the SI gene which encodes for the enzyme sucrase-isomaltase. Sucrase-isomaltase catalyzes the breakdown of sucrose, maltose and larger carbohydrates. Sucrose and maltose are disaccharides, and are broken down into simple sugars during digestion. Sucrose is broken down into glucose and fructose, while maltose is broken down into two glucose molecules. This disorder is characterized by stomach cramps, bloating, excess gas production, and diarrhea after ingestion of sucrose and maltose. These digestive problems can lead to failure to thrive and malnutrition. There is no cure for Sucrase-Isomaltase Deficiency, however orally administrated Sacrosidase can help relieve symptoms. Similarly, restricting high sugar diets can also help. Most affected children are better able to tolerate sucrose and maltose as they get older. Frequency of Sucrase-Isomaltase Deficiency is about 1 in 5,000 with European descent. 

PW122101

Pw122101 View Pathway
disease

Sucrase-Isomaltase Deficiency

Rattus norvegicus
Congenital sucrase-isomaltase deficiency is a rare inborn error of metabolism (IEM) and autosomal recessive disorder caused by mutatins in the SI gene which encodes for the enzyme sucrase-isomaltase. Sucrase-isomaltase catalyzes the breakdown of sucrose, maltose and larger carbohydrates. Sucrose and maltose are disaccharides, and are broken down into simple sugars during digestion. Sucrose is broken down into glucose and fructose, while maltose is broken down into two glucose molecules. This disorder is characterized by stomach cramps, bloating, excess gas production, and diarrhea after ingestion of sucrose and maltose. These digestive problems can lead to failure to thrive and malnutrition. There is no cure for Sucrase-Isomaltase Deficiency, however orally administrated Sacrosidase can help relieve symptoms. Similarly, restricting high sugar diets can also help. Most affected children are better able to tolerate sucrose and maltose as they get older. Frequency of Sucrase-Isomaltase Deficiency is about 1 in 5,000 with European descent. 

PW000533

Pw000533 View Pathway
disease

Sucrase-Isomaltase Deficiency

Homo sapiens
Congenital sucrase-isomaltase deficiency is a rare inborn error of metabolism (IEM) and autosomal recessive disorder caused by mutatins in the SI gene which encodes for the enzyme sucrase-isomaltase. Sucrase-isomaltase catalyzes the breakdown of sucrose, maltose and larger carbohydrates. Sucrose and maltose are disaccharides, and are broken down into simple sugars during digestion. Sucrose is broken down into glucose and fructose, while maltose is broken down into two glucose molecules. This disorder is characterized by stomach cramps, bloating, excess gas production, and diarrhea after ingestion of sucrose and maltose. These digestive problems can lead to failure to thrive and malnutrition. There is no cure for Sucrase-Isomaltase Deficiency, however orally administrated Sacrosidase can help relieve symptoms. Similarly, restricting high sugar diets can also help. Most affected children are better able to tolerate sucrose and maltose as they get older. Frequency of Sucrase-Isomaltase Deficiency is about 1 in 5,000 with European descent. 

PW127331

Pw127331 View Pathway
disease

Sucrase-Isomaltase Deficiency

Homo sapiens
Congenital sucrase-isomaltase deficiency is a rare inborn error of metabolism (IEM) and autosomal recessive disorder caused by mutatins in the SI gene which encodes for the enzyme sucrase-isomaltase. Sucrase-isomaltase catalyzes the breakdown of sucrose, maltose and larger carbohydrates. Sucrose and maltose are disaccharides, and are broken down into simple sugars during digestion. Sucrose is broken down into glucose and fructose, while maltose is broken down into two glucose molecules. This disorder is characterized by stomach cramps, bloating, excess gas production, and diarrhea after ingestion of sucrose and maltose. These digestive problems can lead to failure to thrive and malnutrition. There is no cure for Sucrase-Isomaltase Deficiency, however orally administrated Sacrosidase can help relieve symptoms. Similarly, restricting high sugar diets can also help. Most affected children are better able to tolerate sucrose and maltose as they get older. Frequency of Sucrase-Isomaltase Deficiency is about 1 in 5,000 with European descent. 

PW000841

Pw000841 View Pathway
metabolic

Sucrose Biosynthesis

Solanum lycopersicum
The major product of photosynthesis in most plants, including Solanum lycopersicum, is sucrose. Sucrose is essential for plant development, growth, storage of carbon, stress protection, signal transduction, among other functions. In plants that undergo photosynthesis, D-glyceraldehyde 3-phosphate is transported into the cytoplasm from the chloropast. It is then modified by enzymes involved in gluconeogenesis and transformed to beta-D-fructofuranose 6-phosphate which is then used to produce sucrose. This is done by synthesizing the phosphorylated entity of sucrose, known as sucrose 6F-phosphate, and then dephosphorylating the chemical via the enzyme sucrose-phosphate phosphatase into sucrose. Interestingly, sucrose is synthesized in plants and cyanobacteria, but not in other organisms. Many plants also contain the enzyme sucrose synthase, whose title is misleading because under physiological conditions this enzyme usually catalyzes a sucrose degradation reaction, but under rare circumstances can also generate sucrose as a product.

PW145516

Pw145516 View Pathway
drug action

Sucrose Drug Metabolism Action Pathway

Homo sapiens

PW000423

Pw000423 View Pathway
drug action

Sufentanil Action Pathway

Homo sapiens
Sufentanil is a pharmacologically-active synthetic small molecule derived from fentanyl and belongs to a class of drugs called opioids. Opioids are therapeutically employed to achieve analgesia. Sufentanil’s rapid mechanism of action primarily involves its agonistic effects on mu-type opioid receptors which are inhibitory G-coupled protein receptors and lead to the inhibition of adenylate cyclase and decrease in cAMP production. It also inhibits nociceptive neurotransmitter release and induces membrane hyperpolarization. Analgesia, anesthesia, and respiratory depression are a consequence of remifentanial’s action.

PW144820

Pw144820 View Pathway
drug action

Sufentanil Drug Metabolism Action Pathway

Homo sapiens

PW126550

Pw126550 View Pathway
drug action

Sufentanil Opioid Agonist Action Pathway

Homo sapiens
Sufentanil is an opioid used to induce and maintain anesthesia, to act as an analgesic in labor and delivery, and to treat severe, acute pain. It is used as an adjunct in anesthesia, in balanced anesthesia, and as a primary anesthetic agent. It is administered by the intravenous, epidural and sublingual routes. Sufentanil binds to mu opioid receptors, stimulating the exchange of GTP for GDP on the G-protein complex. As the effector system is adenylate cyclase and cAMP located at the inner surface of the plasma membrane, opioids decrease intracellular cAMP by inhibiting adenylate cyclase. Subsequently, the release of nociceptive neurotransmitters such as GABA is inhibited. Opioids close N-type voltage-operated calcium channels and open calcium-dependent inwardly rectifying potassium channels. This results in hyperpolarization and reduced neuronal excitability. Sufentanil acts at A delta and C pain fibres in the dorsal horn of the spinal cord. By decreasing neurotransmitter action there is less pain transmittance into the spinal cord. This leads to less pain perception.

PW145676

Pw145676 View Pathway
drug action

Sugammadex Drug Metabolism Action Pathway

Homo sapiens