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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.
"Ketoglutaric acid" and "ketoglutarate", when not qualified as α or β, almost always refers respectively to α-ketoglutaric acid or α-ketoglutarate. [2] α-Ketoglutarate is an intermediate in the citric acid cycle, a cycle that supplies the energy to cells. [2] It is also an intermediate in or product of several other metabolic pathways.
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 .
The Mayo Clinic diet is consistently ranked as one of the best diets according to U.S New and World Report's rankings, coming in fourth for the overall best diet in the 2022 rankings.
α-ketoglutarate + NAD + + CoA → Succinyl CoA + CO 2 + NADH Oxoglutarate dehydrogenase (α-Ketoglutarate dehydrogenase) This reaction proceeds in three steps: decarboxylation of α-ketoglutarate, reduction of NAD + to NADH, and subsequent transfer to CoA, which forms the end product, succinyl CoA. ΔG°' for this reaction is -7.2 kcal mol −1.
The Mayo Clinic diet was created by weight management practitioners at the Mayo Clinic and was designed as a lifestyle change program to promote gradual and sustained weight loss, says Melissa ...
HPPD is categorized within a class of oxygenase enzymes that usually utilize α-ketoglutarate and diatomic oxygen to oxygenate or oxidize a target molecule. [5] However, HPPD differs from most molecules in this class due to the fact that it does not use α-ketoglutarate, and it only utilizes two substrates while adding both atoms of diatomic oxygen into the product, homogentisate. [6]
The Autoimmune Protocol (AIP) diet is an elimination diet designed to help reduce inflammation, manage symptoms and improve quality of life for people with autoimmune diseases.