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EC Tree
The enzyme appears in viruses and cellular organisms
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GDP-4-keto-rhamnose reductase
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GDP-6-deoxy-D-talose dehydrogenase
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guanosine diphospho-6-deoxy-D-talose dehydrogenase
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GDP-6-deoxy-alpha-D-talose + NAD(P)+ = GDP-4-dehydro-alpha-D-rhamnose + NAD(P)H + H+
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GDP-6-deoxy-alpha-D-talose:NAD(P)+ 4-oxidoreductase
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GDP-4-dehydro-6-deoxy-D-talose + NAD(P)H
GDP-6-deoxy-D-talose + NAD(P)+
GDP-4-dehydro-6-deoxy-D-talose + NAD(P)H
GDP-6-deoxy-D-talose + NAD(P)+
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GDP-4-dehydro-6-deoxy-D-talose + NAD(P)H
GDP-6-deoxy-D-talose + NAD(P)+
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ir
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GDP-4-dehydro-6-deoxy-D-talose + NAD(P)H
GDP-6-deoxy-D-talose + NAD(P)+
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ir
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3-hydroxy-2-methylbutyryl-coa dehydrogenase deficiency
2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in the HADH2 gene.
3-hydroxy-2-methylbutyryl-coa dehydrogenase deficiency
HSD17B10 replaces HADH2 as the approved designation for the gene mutated in 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency.
3-hydroxy-2-methylbutyryl-coa dehydrogenase deficiency
The reduced expression of the HADH2 protein causes X-linked mental retardation, choreoathetosis, and abnormal behavior.
Mental Retardation, X-Linked
The reduced expression of the HADH2 protein causes X-linked mental retardation, choreoathetosis, and abnormal behavior.
Neurodegenerative Diseases
The reduced expression of the HADH2 protein causes X-linked mental retardation, choreoathetosis, and abnormal behavior.
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expression in Escherichia coli
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gram-negative motile strain GS isolated from soil
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enzyme is identical to 3-hydroxyacyl-CoA dehydrogenase type II
Swissprot
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Uniprot
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cultured skin fibroblast
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TLD_AGGAC
294
0
33342
Swiss-Prot
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A0A5E7ESM1_PSEFL
298
0
32191
TrEMBL
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A0A0M2HEC0_9MICO
321
0
33481
TrEMBL
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A0A238KX35_9RHOB
319
0
34147
TrEMBL
-
A0A833PX26_BURGA
319
0
34351
TrEMBL
-
A0A3N8XSC2_9BURK
296
0
32551
TrEMBL
-
A0A6S7BK11_9BURK
311
0
34302
TrEMBL
-
A0A0H2LV61_VARPD
287
0
31338
TrEMBL
-
A0A833V085_BURGA
270
0
29627
TrEMBL
-
A0A2M9V2T5_BACFG
320
0
35928
TrEMBL
-
A0A0J6ZEX9_MYCCU
317
0
34276
TrEMBL
-
A0A1S8PYX6_CLOBE
277
0
31596
TrEMBL
-
A0A127N1Z0_9PSED
319
0
35384
TrEMBL
-
A0A0M0EBH4_KOMEU
297
0
32432
TrEMBL
-
A0A5E7LM31_PSEFL
298
0
32218
TrEMBL
-
A0A7G6UWG3_9BURK
298
0
32628
TrEMBL
-
A0A1V5YTE2_9BACT
315
0
35424
TrEMBL
-
A0A1X1PCL3_9BURK
320
0
34441
TrEMBL
-
A0A6J5DC62_9BURK
320
0
34386
TrEMBL
-
A0A1E7WIX1_9BURK
305
0
32774
TrEMBL
-
A0A0S4I5B8_9PSED
298
0
31984
TrEMBL
-
A0A1C3JUN0_9GAMM
312
0
34395
TrEMBL
-
A0A7R6ZY53_9PROT
294
0
33726
TrEMBL
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A0A4P6X725_HYDPS
294
0
31714
TrEMBL
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A0A240EP39_9VIBR
314
0
34309
TrEMBL
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A0A1V5MGC6_9FIRM
317
0
35209
TrEMBL
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A0A1E7WHI8_9BURK
304
0
32473
TrEMBL
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A0A7G6V3A0_9BURK
321
0
34343
TrEMBL
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A0A2H5XNX6_9BACT
424
1
48969
TrEMBL
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A0A345DA23_9BURK
293
0
32461
TrEMBL
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A0A1V5IUT8_9BACT
298
0
33695
TrEMBL
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A0A7T7BDM9_RALPI
297
0
32439
TrEMBL
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A0A6J5E1E5_9BURK
292
0
32322
TrEMBL
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A0A6J5FMA4_9BURK
303
0
32990
TrEMBL
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A0A644XLZ8_9ZZZZ
302
0
33314
TrEMBL
other Location (Reliability: 3)
B1FXM8_PARG4
Paraburkholderia graminis (strain ATCC 700544 / DSM 17151 / LMG 18924 / NCIMB 13744 / C4D1M)
303
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33228
TrEMBL
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A0A2N7WY06_9BURK
303
0
33327
TrEMBL
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A0A1C3HF66_SERMA
320
0
35817
TrEMBL
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A0A1Y6MHD6_9GAMM
298
0
33473
TrEMBL
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A0A1J5PRP5_9ZZZZ
311
0
33955
TrEMBL
other Location (Reliability: 5)
A0A6G8NL07_9BURK
341
0
37394
TrEMBL
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A0A0B7DFQ3_PSEFL
298
0
32295
TrEMBL
-
A0A0J6VQ11_9MYCO
317
0
34072
TrEMBL
-
A0A517NRP5_9BACT
338
0
36855
TrEMBL
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A0A1X1PB52_9BURK
297
0
32339
TrEMBL
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A0A6J5CD18_9BURK
303
0
33440
TrEMBL
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A0A5B9QTY6_9BACT
340
0
37286
TrEMBL
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A0A1Y0HN27_9PROT
297
0
33283
TrEMBL
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A0A109LG31_PSEFL
304
0
33490
TrEMBL
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A0A1V5VHU4_9BACT
348
0
38492
TrEMBL
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A0A5E7A7F8_PSEFL
298
0
32420
TrEMBL
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A0A7G2JP57_9GAMM
318
0
35560
TrEMBL
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A0A6J5GYK9_9BURK
302
0
33169
TrEMBL
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A0A2S6S1Q6_9PROT
278
0
32029
TrEMBL
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A0A509E969_9HYPH
294
0
33163
TrEMBL
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A0A6J5BYV6_9BURK
303
0
33237
TrEMBL
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A0A6J5G6T7_9BURK
318
0
34313
TrEMBL
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A0A6J5K741_9BURK
311
0
34350
TrEMBL
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A0A543Q395_ACITH
328
0
35690
TrEMBL
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A0A6S7AZP2_9BURK
302
0
33236
TrEMBL
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A0A1V4WZH6_9DELT
328
0
36583
TrEMBL
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A0A348AGC7_9FIRM
327
0
37048
TrEMBL
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HCD2_BOVIN
261
0
27140
Swiss-Prot
Secretory Pathway (Reliability: 4)
HCD2_HUMAN
261
0
26923
Swiss-Prot
other Location (Reliability: 1)
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28000
x * 28000, SDS-PAGE
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L122C
missence mutation found in patient with inborn error of isoleucine degradation, almost complete loss of enzyme activity
R130C
missence mutation found in patient with inborn error of isoleucine degradation, almost complete loss of enzyme activity
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medicine
L122C and R130C are missence mutations found in patients with inborn error of isoleucine degradation, almost complete loss of enzyme activity
synthesis
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use of enzyme for synthesis of GDP-deoxyhexoses
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Markovitz, A.
Biosynthesis of guanosine diphosphate D-rhamnose and guanosine diphosphate D-talomethylose from guanosine diphosphate alpha-D-mannose
J. Biol. Chem.
239
2091-2098
1964
Bacteria
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Ofman, R.; Ruiter, J.P.; Feenstra, M.; Duran, M.; Poll-The, B.T.; Zschocke, J.; Ensenauer, R.; Lehnert, W.; Sass, J.O.; Sperl, W.; Wanders, R.J.
2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in the HADH2 gene
Am. J. Hum. Genet.
72
1300-1307
2003
Bos taurus (O02691), Bos taurus, Homo sapiens (Q99714), Homo sapiens
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Maki, M.; Jarvinen, N.; Rabina, J.; Maaheimo, H.; Mattila, P.; Renkonen, R.
Cloning and functional expression of a novel GDP-6-deoxy-D-talose synthetase from Actinobacillus actinomycetemcomitans
Glycobiology
13
295-303
2003
Aggregatibacter actinomycetemcomitans
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1.1.1.135 GDP-6-deoxy-D-talose 4-dehydrogenase
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