Homo sapiens (human): 4594
Help
Entry
4594 CDS
T01001
Symbol
MMUT, MCM, MUT
Name
(RefSeq) methylmalonyl-CoA mutase
KO
K01847
methylmalonyl-CoA mutase [EC:
5.4.99.2
]
Organism
hsa
Homo sapiens (human)
Pathway
hsa00280
Valine, leucine and isoleucine degradation
hsa00630
Glyoxylate and dicarboxylate metabolism
hsa00640
Propanoate metabolism
hsa01100
Metabolic pathways
hsa01200
Carbon metabolism
hsa04980
Cobalamin transport and metabolism
Module
hsa_M00741
Propanoyl-CoA metabolism, propanoyl-CoA => succinyl-CoA
Network
nt06024
Valine, leucine and isoleucine degradation
nt06538
Cobalamin transport and metabolism
Element
N00842
Propanoyl-CoA metabolism
N01811
Mitochondrial adenocylation of cobalamin and loading onto MMUT
Disease
H00174
Methylmalonic aciduria
H01400
Secondary hyperammonemia
Brite
KEGG Orthology (KO) [BR:
hsa00001
]
09100 Metabolism
09101 Carbohydrate metabolism
00630 Glyoxylate and dicarboxylate metabolism
4594 (MMUT)
00640 Propanoate metabolism
4594 (MMUT)
09105 Amino acid metabolism
00280 Valine, leucine and isoleucine degradation
4594 (MMUT)
09150 Organismal Systems
09154 Digestive system
04980 Cobalamin transport and metabolism
4594 (MMUT)
Enzymes [BR:
hsa01000
]
5. Isomerases
5.4 Intramolecular transferases
5.4.99 Transferring other groups
5.4.99.2 methylmalonyl-CoA mutase
4594 (MMUT)
BRITE hierarchy
SSDB
Ortholog
Paralog
Gene cluster
GFIT
Motif
Pfam:
MM_CoA_mutase
B12-binding
Motif
Other DBs
NCBI-GeneID:
4594
NCBI-ProteinID:
NP_000246
OMIM:
609058
HGNC:
7526
Ensembl:
ENSG00000146085
UniProt:
P22033
A0A024RD82
B2R6K1
Structure
PDB
LinkDB
All DBs
Position
6:complement(49430360..49463253)
Genome browser
AA seq
750 aa
AA seq
DB search
MLRAKNQLFLLSPHYLRQVKESSGSRLIQQRLLHQQQPLHPEWAALAKKQLKGKNPEDLI
WHTPEGISIKPLYSKRDTMDLPEELPGVKPFTRGPYPTMYTFRPWTIRQYAGFSTVEESN
KFYKDNIKAGQQGLSVAFDLATHRGYDSDNPRVRGDVGMAGVAIDTVEDTKILFDGIPLE
KMSVSMTMNGAVIPVLANFIVTGEEQGVPKEKLTGTIQNDILKEFMVRNTYIFPPEPSMK
IIADIFEYTAKHMPKFNSISISGYHMQEAGADAILELAYTLADGLEYSRTGLQAGLTIDE
FAPRLSFFWGIGMNFYMEIAKMRAGRRLWAHLIEKMFQPKNSKSLLLRAHCQTSGWSLTE
QDPYNNIVRTAIEAMAAVFGGTQSLHTNSFDEALGLPTVKSARIARNTQIIIQEESGIPK
VADPWGGSYMMECLTNDVYDAALKLINEIEEMGGMAKAVAEGIPKLRIEECAARRQARID
SGSEVIVGVNKYQLEKEDAVEVLAIDNTSVRNRQIEKLKKIKSSRDQALAERCLAALTEC
AASGDGNILALAVDASRARCTVGEITDALKKVFGEHKANDRMVSGAYRQEFGESKEITSA
IKRVHKFMEREGRRPRLLVAKMGQDGHDRGAKVIATGFADLGFDVDIGPLFQTPREVAQQ
AVDADVHAVGISTLAAGHKTLVPELIKELNSLGRPDILVMCGGVIPPQDYEFLFEVGVSN
VFGPGTRIPKAAVQVLDDIEKCLEKKQQSV
NT seq
2253 nt
NT seq
+upstream
nt +downstream
nt
atgttaagagctaagaatcagctttttttactttcacctcattacctgaggcaggtaaaa
gaatcatcaggctccaggctcatacagcaacgacttctacaccagcaacagccccttcac
ccagaatgggctgccctggctaaaaagcagctgaaaggcaaaaacccagaagacctaata
tggcacaccccggaagggatctctataaaacccttgtattccaagagagatactatggac
ttacctgaagaacttccaggagtgaagccattcacacgtggaccatatcctaccatgtat
acctttaggccctggaccatccgccagtatgctggttttagtactgtggaagaaagcaat
aagttctataaggacaacattaaggctggtcagcagggattatcagttgcctttgatctg
gcgacacatcgtggctatgattcagacaaccctcgagttcgtggtgatgttggaatggct
ggagttgctattgacactgtggaagataccaaaattctttttgatggaattcctttagaa
aaaatgtcagtttccatgactatgaatggagcagttattccagttcttgcaaattttata
gtaactggagaagaacaaggtgtacctaaagagaagcttactggtaccatccaaaatgat
atactaaaggaatttatggttcgaaatacatacatttttcctccagaaccatccatgaaa
attattgctgacatatttgaatatacagcaaagcacatgccaaaatttaattcaatttca
attagtggataccatatgcaggaagcaggggctgatgccattctggagctggcctatact
ttagcagatggattggagtactctagaactggactccaggctggcctgacaattgatgaa
tttgcaccaaggttgtctttcttctggggaattggaatgaatttctatatggaaatagca
aagatgagagctggtagaagactctgggctcacttaatagagaaaatgtttcagcctaaa
aactcaaaatctcttcttctaagagcacactgtcagacatctggatggtcacttactgag
caggatccctacaataatattgtccgtactgcaatagaagcaatggcagcagtatttgga
gggactcagtctttgcacacaaattcttttgatgaagctttgggtttgccaactgtgaaa
agtgctcgaattgccaggaacacacaaatcatcattcaagaagaatctgggattcccaaa
gtggctgatccttggggaggttcttacatgatggaatgtctcacaaatgatgtttatgat
gctgctttaaagctcattaatgaaattgaagaaatgggtggaatggccaaagctgtagct
gagggaatacctaaacttcgaattgaagaatgtgctgcccgaagacaagctagaatagat
tctggttctgaagtaattgttggagtaaataagtaccagttggaaaaagaagacgctgta
gaagttctggcaattgataatacttcagtgcgaaacaggcagattgaaaaacttaagaag
atcaaatccagcagggatcaagctttggctgaacgttgtcttgctgcactaaccgaatgt
gctgctagcggagatggaaatatcctggctcttgcagtggatgcatctcgggcaagatgt
acagtgggagaaatcacagatgccctgaaaaaggtatttggtgaacataaagcgaatgat
cgaatggtgagtggagcatatcgccaggaatttggagaaagtaaagagataacatctgct
atcaagagggttcataaattcatggaacgtgaaggtcgcagacctcgtcttcttgtagca
aaaatgggacaagatggccatgacagaggagcaaaagttattgctacaggatttgctgat
cttggttttgatgtggacataggccctcttttccagactcctcgtgaagtggcccagcag
gctgtggatgcggatgtgcatgctgtgggcataagcaccctcgctgctggtcataaaacc
ctagttcctgaactcatcaaagaacttaactcccttggacggccagatattcttgtcatg
tgtggaggggtgataccacctcaggattatgaatttctgtttgaagttggtgtttccaat
gtatttggtcctgggactcgaattccaaaggctgccgttcaggtgcttgatgatattgag
aagtgtttggaaaagaagcagcaatctgtataa
COMPOUND: C00194
Help
Entry
C00194 Compound
Name
Cobamide coenzyme;
Adenosylcobalamin;
Adenosylcob(III)alamin;
Deoxyadenosylcobalamin;
Cobamamide;
Vitamin B12 coenzyme;
5,6-Dimethylbenzimidazolyl-5-deoxyadenosyl-cobamide;
(5'-Deoxy-5'-adenosyl)cobamide coenzyme;
(5,6-Dimethylbenzimidazolyl)cobamide coenzyme;
alpha-(5,6-Dimethylbenzimidazolyl)cobamide coenzyme;
5'-Deoxy-5'-adenosylcobalamin;
5'-Deoxy-5'-adenosyl vitamin B12;
5'-Deoxy-5'-adenosyl-5,6-dimethylbenzimidazolylcobamide;
5,6-Dimethylbenzimidazolyl-Co-5'-deoxy-5'-adenosylcobamide;
Calomide;
Cobalamin coenzyme;
Coenzyme B12;
DMBC coenzyme;
Dibencozide;
Funacomide
Formula
C72H100CoN18O17P
Exact mass
1578.6583
Mol weight
1579.5818
Structure
Mol file
KCF file
DB search
Remark
Same as:
D00042
Comment
Coordination compound
Representative chemical structure of Cobamide [CPD:
C00210
].
Reaction
R01492
R05223
R11173
R12183
R12857
R12945
Pathway
map00860
Porphyrin metabolism
map01100
Metabolic pathways
map01240
Biosynthesis of cofactors
map04977
Vitamin digestion and absorption
map04980
Cobalamin transport and metabolism
Module
M00122
Cobalamin biosynthesis, cobyrinate a,c-diamide => cobalamin
Network
nt06538
Cobalamin transport and metabolism
Enzyme
2.5.1.17
2.5.1.154
2.7.8.26
3.1.3.73
Brite
Compounds with biological roles [BR:
br08001
]
Vitamins and cofactors
Cofactors
Coenzymes
C00194 Cobamamide
Anatomical Therapeutic Chemical (ATC) classification [BR:
br08303
]
B BLOOD AND BLOOD FORMING ORGANS
B03 ANTIANEMIC PREPARATIONS
B03B VITAMIN B12 AND FOLIC ACID
B03BA Vitamin B12 (cyanocobalamin and analogues)
B03BA04 Cobamamide
D00042 Cobamamide (JAN/INN) <JP>
Therapeutic category of drugs in Japan [BR:
br08301
]
3 Agents affecting metabolism
31 Vitamins
313 Vitamin B preparations
3136 Vitamin B12 preparations
D00042 Cobamamide (JAN/INN)
BRITE hierarchy
Other DBs
CAS:
13870-90-1
PubChem:
3494
ChEBI:
18408
NIKKAJI:
J2.221.278H
LinkDB
All DBs
KCF data
Show
ATOM 109
1 Z Co 19.1366 -15.4147 #+
2 N2x N 17.8076 -14.1535
3 N1y N 17.8076 -16.8112
4 N2x N 20.5104 -14.1985
5 N2x N 20.4804 -16.7811
6 N5x N 21.2504 -25.5382
7 C1z C 16.5841 -14.3485
8 C2y C 18.0254 -12.9373
9 C1y C 16.5841 -16.6309
10 C2y C 18.0103 -18.0348
11 C2y C 20.2476 -12.9747
12 C2y C 21.7566 -14.3111
13 C2y C 20.2627 -17.9897
14 C2y C 21.7266 -16.6159
15 C8y C 22.4141 -25.9061
16 C8x C 20.5371 -26.5293
17 C1z C 16.0208 -13.2299
18 C1a C 15.3677 -14.6790
19 C1y C 16.9143 -12.3590
20 C2y C 19.1440 -12.5169
21 C1y C 16.0208 -17.7345
22 C1z C 16.9143 -18.6130
23 C2y C 19.1440 -18.4702
24 C1z C 21.3288 -12.3590
25 C1y C 22.2522 -13.1774
26 C2x C 22.2147 -15.4598
27 C1y C 21.3438 -18.5830
28 C1z C 22.2522 -17.7345
29 C8y C 22.4065 -27.1299
30 C8x C 23.4652 -25.3055
31 N4y N 21.2504 -27.5127
32 C1b C 13.1722 -13.2225
33 C1a C 15.6905 -12.0213
34 C1b C 16.9368 -11.1204
35 C1a C 19.1366 -11.2630
36 C1b C 14.6845 -17.8621
37 C1b C 16.5493 -20.8242
38 C1a C 17.8131 -21.3707
39 C1a C 19.1366 -19.7090
40 C1b C 21.3212 -11.1054
41 C1a C 19.9174 -10.0064
42 C1b C 23.4984 -13.1700
43 C1b C 21.4714 -19.8216
44 C1a C 23.4533 -17.4193
45 C1a C 22.7100 -18.9056
46 C8x C 23.4652 -27.7380
47 C8y C 24.5238 -25.9061
48 C1y C 15.6586 -29.6958
49 C5a C 12.5641 -12.1489
50 C1b C 15.8708 -10.4973
51 C5a C 14.2490 -19.0108
52 C1b C 15.3281 -21.1673
53 C5a C 22.3948 -10.4823
54 C1b C 24.0991 -12.0888
55 C1b C 22.5975 -20.3321
56 C8y C 24.5238 -27.1299
57 C1a C 25.5823 -25.3055
58 C1y C 15.2907 -28.5247
59 O2x O 14.5999 -30.2965
60 N1a N 11.3253 -12.1339
61 O5a O 13.1872 -11.0827
62 C5a C 15.8858 -9.2511
63 N1a N 13.0254 -19.2135
64 O5a O 15.0300 -19.9568
65 C5a C 15.3207 -22.4136
66 N1a N 22.3874 -9.2361
67 O5a O 23.4760 -11.0978
68 C5a C 25.3453 -12.0814
69 C5a C 22.7177 -21.5633
70 C1a C 25.5823 -27.7380
71 C1y C 14.0595 -28.5097
72 O1a O 16.0189 -27.5487
73 C1y C 13.6841 -29.6658
74 N1a N 14.8123 -8.6277
75 O5a O 16.9668 -8.6428
76 N1b N 14.2471 -23.0367
77 O5a O 16.3793 -23.0443
78 N1a N 25.9610 -11.0002
79 O5a O 25.9758 -13.1624
80 N1a N 21.7042 -22.2766
81 O5a O 23.8587 -22.0815
82 O2b O 13.3536 -27.5112
83 C1b C 12.4303 -30.0862
84 C1b C 14.2471 -24.3206
85 P1b P 11.9948 -26.9106
86 O1a O 12.1751 -31.3775
87 C1c C 13.1285 -24.9661
88 O2b O 13.1359 -26.2500
89 O1c O 10.8538 -26.2500 #-
90 O1c O 11.0639 -27.8415
91 C1a C 12.0249 -24.3206
92 C1y C 26.2373 -16.0301
93 O2x O 27.2582 -15.2868
94 C1y C 26.6350 -17.2313
95 C1y C 28.3093 -16.0752
96 C1y C 27.9113 -17.2313
97 O1a O 26.2222 -18.4474
98 N4y N 29.4954 -15.6922
99 O1a O 28.5196 -18.2824
100 C8y C 30.4791 -16.3754
101 C8x C 29.9385 -14.4009
102 C8y C 31.6202 -15.5346
103 N5x N 30.5991 -17.6893
104 N5x N 31.2749 -14.4085
105 C8y C 32.7839 -16.0525
106 C8x C 31.8303 -18.2297
107 N5x N 32.9339 -17.4189
108 N1a N 33.8048 -15.2868
109 C1b C 24.9159 -14.7656
BOND 119
1 1 3 1
2 2 7 1
3 2 8 2
4 3 9 1
5 3 10 1
6 4 11 1
7 4 12 2
8 5 13 2
9 5 14 1
10 6 15 1
11 6 16 2
12 7 17 1
13 7 18 1 #Down
14 8 19 1
15 8 20 1
16 9 21 1
17 10 22 1
18 10 23 2
19 11 24 1
20 12 25 1
21 12 26 1
22 13 27 1
23 14 28 1
24 15 29 2
25 15 30 1
26 16 31 1
27 17 32 1 #Up
28 17 33 1 #Down
29 19 34 1 #Down
30 20 35 1
31 21 36 1 #Up
32 22 37 1 #Down
33 22 38 1 #Up
34 23 39 1
35 24 40 1 #Up
36 24 41 1 #Down
37 25 42 1 #Down
38 27 43 1 #Down
39 28 44 1
40 28 45 1
41 29 46 1
42 30 47 2
43 48 31 1 #Up
44 32 49 1
45 34 50 1
46 36 51 1
47 37 52 1
48 40 53 1
49 42 54 1
50 43 55 1
51 46 56 2
52 47 57 1
53 48 58 1
54 48 59 1
55 49 60 1
56 49 61 2
57 50 62 1
58 51 63 1
59 51 64 2
60 52 65 1
61 53 66 1
62 53 67 2
63 54 68 1
64 55 69 1
65 56 70 1
66 58 71 1
67 58 72 1 #Up
68 73 59 1
69 62 74 1
70 62 75 2
71 65 76 1
72 65 77 2
73 68 78 1
74 68 79 2
75 69 80 1
76 69 81 2
77 71 82 1 #Up
78 73 83 1 #Down
79 76 84 1
80 82 85 1
81 83 86 1
82 84 87 1
83 85 88 1
84 85 89 1
85 85 90 2
86 87 91 1 #Down
87 7 9 1
88 11 20 2
89 13 23 1
90 14 26 2
91 17 19 1
92 21 22 1
93 24 25 1
94 27 28 1
95 29 31 1
96 47 56 1
97 71 73 1
98 87 88 1
99 92 93 1
100 92 94 1
101 93 95 1
102 94 96 1
103 94 97 1 #Down
104 95 98 1 #Up
105 96 99 1 #Down
106 98 100 1
107 98 101 1
108 100 102 2
109 100 103 1
110 101 104 2
111 102 105 1
112 103 106 2
113 105 107 2
114 105 108 1
115 95 96 1
116 102 104 1
117 106 107 1
118 92 109 1 #Up
119 1 109 1
» Japanese version
DBGET
integrated database retrieval system
