| lysinuric protein intolerance | An autosomal recessive disorder characterised by elevated levels of dibasic amino acids (e.g., l-lysine, l-arginine, and l-ornithine) in the urine; apparently due to a defect in dibasic amino acid transport. (05 Mar 2000) |
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| glucose intolerance | A pathological state in which the fasting plasma glucose level is less than 140 mg per deciliter and the 30-, 60-, or 90-minute plasma glucose concentration following a glucose tolerance test exceeds 200 mg per deciliter. This condition is seen frequently in diabetes mellitus but also occurs with other diseases. (12 Dec 1998) |
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| hereditary fructose intolerance | A metabolic error due to deficiency of hepatic fructose 1,6-bisphosphate aldolase B (which also acts on fructose 1-phosphate); the second enzyme in the specific fructose pathway; vomiting and hypoglycaemia follow ingestion of fructose; prolonged fructose ingestion in young children results in failure to thrive and in jaundice, hepatomegaly, albuminuria, aminoaciduria, and sometimes cachexia and death; autosomal recessive inheritance in most families. (05 Mar 2000) |
| intolerance | Inability to withstand, sensitivity, as to a drug. Origin: L. Tolerare = to bear (18 Nov 1997) |
| lactose intolerance | A disorder characterised by abdominal cramps and diarrhoea after the consumption of food containing lactose (for example milk, ice cream), believed to occur due to a deficiency of intestinal lactase (enzyme that breaks down lactose), may appear first in young adults who have previously tolerated milk well as infants. (27 Sep 1997) |
| fructose intolerance | An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (ec 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycaemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet. (12 Dec 1998) |
| acetoacetyl-acyl carrier protein synthase | <enzyme> E coli enzyme, that catalyses condensation of malonyl-acyl carrier protein plus acetyl-acyl carrier protein; not inhibited by cerulenin Registry number: EC 2.3.1.- Synonym: acetoacetyl-acp synthase (26 Jun 1999) |
| acid soluble spore protein | <molecular biology> A DNA binding protein in the spores of some bacteria, thought to stabilise the DNA in an A configuration, so protecting it from cleavage by enzymes or UV light. (18 Nov 1997) |
| acute-phase protein | <haematology> These plasma proteins (in addition to fibrinogen) increase 25% or more in response to inflammation and injury are under direct control of interleukin-6 (IL-6) (hepatocyte-stimulating factor). Other proteins which increase are ceruloplasmin, C3 and C4 which increase 50% or more; alpha-1 acid glycoprotein, alpha-1 antitrypsin, haptoglobin and fibrinogen (the major determinant of viscosity 1 ) which increase two- to fourfold; C-reactive protein (CRP) and serum amyloid A which increase several hundred-fold. Despite long-held clinical opinion to the contrary, available data indicate that neither ESR nor measurement of specific acute-phase reactants are useful in excluding underlying infection or inflammation regardless of the pretest probability. These proteins are secreted into the blood in increased or decreased quantities by hepatocytes in response to trauma, inflammation, or disease. They can serve as inhibitors or mediators of the inflammatory processes. Certain acute-phase proteins have been used to diagnose and follow the course of diseases or as tumour markers. See also: amyloid, c-reactive protein, erythrocyte sedimentation rate, viscosity. (25 Jun 1999) |
| acyl-(acyl-carrier-protein)-phospholipid acyltransferase | <enzyme> Catalyses the formation of phosphatidylethanolamine from acyl-acyl carrier protein and 2-acyl-sn-glycero-3-phosphoethanolamine Registry number: EC 2.3.1.40 Synonym: 2-acyl-gpe acyltransferase, 2-acylglycerophosphoethanolamine acyltransferase (26 Jun 1999) |
| acyl-(acyl-carrier-protein)-UDP-N-acetylglucosamine acyltransferase | <enzyme> E coli enzyme involved in lipid a biosynthesis; uses beta-hydroxymyristoyl-acyl carrier protein to form udp-3-monoacyl-n-acetylglucosamine; amino acid sequence given in second source Registry number: EC 2.3.1.129 Synonym: udp-aguatransferase, lpxa protein, udp-n-acetylglucosamine-3-acyltransferase, udp-n-acetylglucosamine 3-o-acyltransferase, udp-3-o-(r-3-hydroxymyristoyl)glucosamine-n-acyltransferase, lpxd protein, fira gene product, fira protein (26 Jun 1999) |
| acyl carrier protein | <protein> A small (77 peptides long) protein which binds six other enzymes involved in fatty acid synthesis. It was first isolated in E. Coli bacteria. (09 Oct 1997) |
| acyl carrier protein acylase | <enzyme> From E coli Registry number: EC 2.3.1.- Synonym: acp acylase (26 Jun 1999) |
| acyl protein synthetase | <enzyme> Component of the fatty acid reductase complex of luminescent bacteria Registry number: EC 2.3.1.- Synonym: luxe gene product, fatty acyl-protein synthetase (26 Jun 1999) |
| AKT1 protein kinase | <enzyme> Human homolog of v-akt oncogene product Registry number: EC 2.7.10.- Synonym: akt1 protein, human (26 Jun 1999) |
| AMP-activated protein kinase kinase | <enzyme> An endogenous kinase kinase; reactivates the inactive form of AMP-activated protein kinase (AMP-pk); phosphorylates the 63-kD subunit of AMP-pk Registry number: EC 2.7.1.- Synonym: AMP-pk reactivator, hmg CoA reductase kinase kinase (26 Jun 1999) |