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Items: 14

1.

Cobalamin (Cbl, vitamin B12) transport and metabolism

Vitamin B12 (cobalamin, Cbl) is a water-soluble vitamin with a key role in blood formation and normal functioning of the brain and nervous system. Cbl consists of a planar corrin ring coordinating with a cobalt atom through four nitrogen atoms. A 5,6-dimethylbenzamidizole base coordinates...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
2.

Propionyl-CoA catabolism

Propionyl-CoA is a product of the catabolism of the amino acids, leucine, methionine, and threonine, and of the beta-oxidation of fatty acids with odd numbers of carbon atoms. The three reactions of this pathway convert propionyl-CoA to succinyl-CoA, an intermediate of the citric...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
3.

Mitochondrial Fatty Acid Beta-Oxidation

Beta-oxidation begins once fatty acids have been imported into the mitochondrial matrix by carnitine acyltransferases. The beta-oxidation spiral of fatty acids metabolism involves the repetitive removal of two carbon units from the fatty acyl chain. There are four steps to this process:...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
4.

Defective MUT causes methylmalonic aciduria mut type

Defects in MUT cause methylmalonic aciduria, mut type (MMAM; MIM:251000), an often fatal disorder of organic acid metabolism (Worgan et al. 2006).

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
5.

Defective MMAA causes methylmalonic aciduria type cblA

Defects in MMAA cause methylmalonic aciduria type cblA (cblA aka methylmalonic aciduria type A or vitamin B12-responsive methylmalonic aciduria of cblA complementation type; MIM:251100). Affected individuals accumulate methylmalonic acid in the blood and urine and are prone to potentially...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
6.

Defects in cobalamin (B12) metabolism

Cobalamin (Cbl, vitamin B12) is a nutrient essential for normal functioning of the brain and nervous system and for the formation of blood. Cbl-dependent methionine synthase (MTR) is required for conversion of 5-methyltetrahydrofolate (metTHF) to tetrahydrofolate (THF), in addition...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
7.

Metabolism of water-soluble vitamins and cofactors

Vitamins are a diverse group of organic compounds, required in small amounts in the diet. They have distinct biochemical roles, often as coenzymes, and are either not synthesized or synthesized only in limited amounts by human cells. Vitamins are classified according to their solubility,...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
8.

Diseases of metabolism

Metabolic processes in human cells generate energy through the oxidation of molecules consumed in the diet and mediate the synthesis of diverse essential molecules not taken in the diet as well as the inactivation and elimination of toxic ones generated endogenously or present in...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
9.

Defects in vitamin and cofactor metabolism

Vitamins are essential nutrients, required in small amounts from the diet for the normal growth and development of a multicellular organism. Where there is vitamin deficiency, either by poor diet or a defect in metabolic conversion, diseases called Avitaminoses occur. Currently, cobalamin...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
10.

Metabolism of vitamins and cofactors

Vitamins are a diverse group of organic compounds, classified according to their solubility, either fat-soluble or water-soluble, that are either not synthesized or synthesized only in limited amounts by human cells. They are required in small amounts in the diet and have distinct...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
11.

Fatty acid, triacylglycerol, and ketone body metabolism

The reactions involved in the metabolism of fatty acids and of the triacylglycerols and ketone bodies derived from them form a closely interrelated, coordinately regulated module that plays a central role in human energy metabolism.

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
12.

Metabolism of lipids and lipoproteins

Lipids are hydrophobic but otherwise chemically diverse molecules that play a wide variety of roles in human biology. They include ketone bodies, fatty acids, triacylglycerols, phospholipids and sphingolipids, eicosanoids, cholesterol, bile salts, steroid hormones, and fat-soluble...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
13.

Metabolism

Metabolic processes in human cells generate energy through the oxidation of molecules consumed in the diet and mediate the synthesis of diverse essential molecules not taken in the diet as well as the inactivation and elimination of toxic ones generated endogenously or present in...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens
14.

Disease

Biological processes are captured in Reactome by identifying the molecules (DNA, RNA, protein, small molecules) involved in them and describing the details of their interactions. From this molecular viewpoint, human disease pathways have three mechanistic causes: the inclusion of...

Type
:
pathway
Taxonomic scope
:
organism-specific biosystem
Organism
:
Homo sapiens

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