Disease relevance of C01188

• We show that HBDH from Rhodopseudomonas spheroides (BCL, grade II) contains a 3-hydroxyisobutyrate dehydrogenase (HIBDH) activity: activity with 3-hydroxyisobutyrate as substrate was greater than 10% of that with 3-hydroxybutyrate [1].
• Transformed E. coli cells overproduced 3-hydroxyisobutyrate dehydrogenase, and the recombinant enzyme was purified to homogeneity with a high yield [2].

High impact information on C01188

• Essential sulfhydryl groups may be present at both the 3-hydroxyisobutyrate and NAD+ binding sites since inhibition by sulfhydryl-binding agents was differentially blocked by each substrate [3].
• A kinetic study confirms that this enzyme has strict substrate specificity for 3-hydroxyisobutyrate and serine, but it cannot distinguish the chirality of the substrates [4].
• Contribution of 3-hydroxyisobutyrate to the measurement of 3-hydroxybutyrate in human plasma: comparison of enzymatic and gas-liquid chromatography-mass spectrometry assays in normal and in diabetic subjects [5].
• Valine metabolism. Gluconeogenesis from 3-hydroxyisobutyrate [6].
• 3-Hydroxyisobutyrate is a good gluconeogenic substrate in isolated cortical tubules and hepatocytes [6].

Biological context of C01188

• The level of 3-hydroxyisobutyrate in stored samples of midtrimester amniotic fluid was found to be high [7].

Anatomical context of C01188

• The conversion of 1-14C-leucine and 1-14C-valine to 14CO2 and of U-L-14C-valine to its major metabolic product 3-hydroxyisobutyric acid by fibroblasts derived from the patient was less than 5% of control [8].

Associations of C01188 with other chemical compounds

• The maximal rate of gluconeogenesis from 3-hydroxyisobutyrate was greater than from 2-oxoisovalerate [6].
• The excretion of 3-hydroxyisobutyric acid ranged from 170 to 390 mmol/mol of creatinine [9].

Gene context of C01188

• The pathway is effectively truncated at the level of 3-hydroxyisobutyrate because of very low expression of two enzymes required for the complete pathway, 3-hydroxyisobutyrate dehydrogenase and methylmalonate semialdehyde dehydrogenase [10].

References