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α-Ketoglutarate is a component of the citric acid cycle, a cyclical metabolic pathway located in the mitochondria.This cycle supplies the energy that cells need by sequentially metabolizing (indicated by →) citrate through seven intermediate metabolites and then converting the eighth intermediate metabolite, oxaloacetate, back to citrate: [2]
Hyperlysinemia has an autosomal recessive pattern of inheritance. Hyperlysinemia is inherited in an autosomal recessive manner. [2] This means the defective gene responsible for the disorder is located on an autosome, and two copies of the defective gene (one inherited from each parent) are required in order to be born with the disorder.
A deficiency in any of the enzymes of this complex as well as an inhibition of the complex as a whole leads to a buildup of branched-chain amino acids and their harmful derivatives in the body. These accumulations lend a sweet smell to bodily excretions (such as ear wax and urine), leading to a pathology known as maple syrup urine disease. [19]
Calcium-alpha-ketoglutarate (C 5 H 4 CaO 5 •H 2 O) is a special form mineral calcium that can be used to restore calcium concentration level in the blood back to normal. Calcium-alpha-ketoglutarate binds excess phosphate and pass it as a waste, re-establishing normal balance of calcium and phosphate in the body.
[1] [2] In these situations they decrease the need for blood transfusions. [2] The different agents are more or less equivalent. [2] They are given by injection. [2] Common side effects may include joint pain, rash, vomiting, and headache. [4] Serious side effects may include heart attacks, stroke, increased cancer growth, or pure red cell ...
In about 10% of cases the blood sugar is not significantly elevated ("euglycemic diabetic ketoacidosis"). [3] A pH measurement is performed to detect acidosis. Blood from a vein is adequate, as there is little difference between the arterial and the venous pH; arterial samples are only required if there are concerns about oxygen levels. [6]
Ketotic hypoglycemia refers to any circumstance in which low blood glucose is accompanied by ketosis, the presence of ketone bodies (such as beta-hydroxybutyrate) in the blood or urine. This state can be either physiologic or pathologic; physiologic ketotic hypoglycemia is a common cause of hypoglycemia in children, often in response to ...
Ferric chloride test (detects abnormal metabolites in urine) Ninhydrin paper chromatography (detects abnormal amino acid patterns) Guthrie test (detects excessive amounts of specific amino acids in blood) The dried blood spot can be used for multianalyte testing using Tandem Mass Spectrometry (MS/MS). This given an indication for a disorder.