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3-metyl-2-oxobutanoátdehydrogenáza (lipoamid)
SYS d042942 LBL 00000nz--a2200000o--4500 005 20250606214156.3 008 030709|||anznnbabn-----------|-a|a------ 040 $b slo $a DNLM $d BA006 065 $a D08.811.682.657.350.760 066 $a 01 $c 03 150 $a 3-metyl-2-oxobutanoátdehydrogenáza (lipoamid) $x AD $x AE $x AI $x AN $x BI $x BL $x CF $x CH $x CL $x CS $x DE $x DF $x EC $x GE $x HI $x IM $x IP $x ME $x PD $x PH $x PK $x PO $x RE $x SD $x ST $x TO $x TU $x UL $x UR $2 slo 450 $w v $a 2-Oxoisovalerate Dehydrogenase (Lipoamide) $2 eng 450 $w v $a 2-oxoizovalerátdehydrogenáza (lipoamid) $2 slo 550 $7 sllk_us_auth*d008375 $Y Maple Syrup Urine Disease $w p $a leucinóza 665 $a 2004; use 3-METHYL-2-OXBUTANOATE DEHYDROGENASE (LIPOAMIDE) (NM) 1973-2003 $2 eng 665 $a Amino Acids, Branched Chain (1979-1981) $2 eng 665 $a Carboxy-Lyases (1974-1979) $2 eng 665 $a Keto Acids (1973-1981) $2 eng 665 $a Ketone Oxidoreductases (1982-2003) $2 eng 665 $a Multienzyme Complexes (1982-2003) $2 eng 680 9-
$i A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3. $2 eng 750 -2
$a 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) $2 eng 980 $x M
Number of the records: 1