6DV2

Crystal Structure of Human Mitochondrial Trifunctional Protein

Functional Information from GO Data

Chain	GOid	namespace	contents
A	0003723	molecular_function	RNA binding
A	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
A	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
A	0003988	molecular_function	acetyl-CoA C-acyltransferase activity
A	0004300	molecular_function	enoyl-CoA hydratase activity
A	0005515	molecular_function	protein binding
A	0005654	cellular_component	nucleoplasm
A	0005739	cellular_component	mitochondrion
A	0005740	cellular_component	mitochondrial envelope
A	0005741	cellular_component	mitochondrial outer membrane
A	0005743	cellular_component	mitochondrial inner membrane
A	0005783	cellular_component	endoplasmic reticulum
A	0006629	biological_process	lipid metabolic process
A	0006631	biological_process	fatty acid metabolic process
A	0006635	biological_process	fatty acid beta-oxidation
A	0010467	biological_process	gene expression
A	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
A	0016740	molecular_function	transferase activity
A	0016746	molecular_function	acyltransferase activity
A	0016747	molecular_function	acyltransferase activity, transferring groups other than amino-acyl groups
A	0042645	cellular_component	mitochondrial nucleoid
A	0044877	molecular_function	protein-containing complex binding
A	0050633	molecular_function	acetyl-CoA C-myristoyltransferase activity
A	0071222	biological_process	cellular response to lipopolysaccharide
A	0097228	cellular_component	sperm principal piece
A	0106222	molecular_function	lncRNA binding
B	0003723	molecular_function	RNA binding
B	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
B	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
B	0003988	molecular_function	acetyl-CoA C-acyltransferase activity
B	0004300	molecular_function	enoyl-CoA hydratase activity
B	0005515	molecular_function	protein binding
B	0005654	cellular_component	nucleoplasm
B	0005739	cellular_component	mitochondrion
B	0005740	cellular_component	mitochondrial envelope
B	0005741	cellular_component	mitochondrial outer membrane
B	0005743	cellular_component	mitochondrial inner membrane
B	0005783	cellular_component	endoplasmic reticulum
B	0006629	biological_process	lipid metabolic process
B	0006631	biological_process	fatty acid metabolic process
B	0006635	biological_process	fatty acid beta-oxidation
B	0010467	biological_process	gene expression
B	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
B	0016740	molecular_function	transferase activity
B	0016746	molecular_function	acyltransferase activity
B	0016747	molecular_function	acyltransferase activity, transferring groups other than amino-acyl groups
B	0042645	cellular_component	mitochondrial nucleoid
B	0044877	molecular_function	protein-containing complex binding
B	0050633	molecular_function	acetyl-CoA C-myristoyltransferase activity
B	0071222	biological_process	cellular response to lipopolysaccharide
B	0097228	cellular_component	sperm principal piece
B	0106222	molecular_function	lncRNA binding
C	0003723	molecular_function	RNA binding
C	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
C	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
C	0003988	molecular_function	acetyl-CoA C-acyltransferase activity
C	0004300	molecular_function	enoyl-CoA hydratase activity
C	0005515	molecular_function	protein binding
C	0005654	cellular_component	nucleoplasm
C	0005739	cellular_component	mitochondrion
C	0005740	cellular_component	mitochondrial envelope
C	0005741	cellular_component	mitochondrial outer membrane
C	0005743	cellular_component	mitochondrial inner membrane
C	0005783	cellular_component	endoplasmic reticulum
C	0006629	biological_process	lipid metabolic process
C	0006631	biological_process	fatty acid metabolic process
C	0006635	biological_process	fatty acid beta-oxidation
C	0010467	biological_process	gene expression
C	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
C	0016740	molecular_function	transferase activity
C	0016746	molecular_function	acyltransferase activity
C	0016747	molecular_function	acyltransferase activity, transferring groups other than amino-acyl groups
C	0042645	cellular_component	mitochondrial nucleoid
C	0044877	molecular_function	protein-containing complex binding
C	0050633	molecular_function	acetyl-CoA C-myristoyltransferase activity
C	0071222	biological_process	cellular response to lipopolysaccharide
C	0097228	cellular_component	sperm principal piece
C	0106222	molecular_function	lncRNA binding
D	0003723	molecular_function	RNA binding
D	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
D	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
D	0003988	molecular_function	acetyl-CoA C-acyltransferase activity
D	0004300	molecular_function	enoyl-CoA hydratase activity
D	0005515	molecular_function	protein binding
D	0005654	cellular_component	nucleoplasm
D	0005739	cellular_component	mitochondrion
D	0005740	cellular_component	mitochondrial envelope
D	0005741	cellular_component	mitochondrial outer membrane
D	0005743	cellular_component	mitochondrial inner membrane
D	0005783	cellular_component	endoplasmic reticulum
D	0006629	biological_process	lipid metabolic process
D	0006631	biological_process	fatty acid metabolic process
D	0006635	biological_process	fatty acid beta-oxidation
D	0010467	biological_process	gene expression
D	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
D	0016740	molecular_function	transferase activity
D	0016746	molecular_function	acyltransferase activity
D	0016747	molecular_function	acyltransferase activity, transferring groups other than amino-acyl groups
D	0042645	cellular_component	mitochondrial nucleoid
D	0044877	molecular_function	protein-containing complex binding
D	0050633	molecular_function	acetyl-CoA C-myristoyltransferase activity
D	0071222	biological_process	cellular response to lipopolysaccharide
D	0097228	cellular_component	sperm principal piece
D	0106222	molecular_function	lncRNA binding
E	0003723	molecular_function	RNA binding
E	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
E	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
E	0003988	molecular_function	acetyl-CoA C-acyltransferase activity
E	0004300	molecular_function	enoyl-CoA hydratase activity
E	0005515	molecular_function	protein binding
E	0005654	cellular_component	nucleoplasm
E	0005739	cellular_component	mitochondrion
E	0005740	cellular_component	mitochondrial envelope
E	0005741	cellular_component	mitochondrial outer membrane
E	0005743	cellular_component	mitochondrial inner membrane
E	0005783	cellular_component	endoplasmic reticulum
E	0006629	biological_process	lipid metabolic process
E	0006631	biological_process	fatty acid metabolic process
E	0006635	biological_process	fatty acid beta-oxidation
E	0010467	biological_process	gene expression
E	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
E	0016740	molecular_function	transferase activity
E	0016746	molecular_function	acyltransferase activity
E	0016747	molecular_function	acyltransferase activity, transferring groups other than amino-acyl groups
E	0042645	cellular_component	mitochondrial nucleoid
E	0044877	molecular_function	protein-containing complex binding
E	0050633	molecular_function	acetyl-CoA C-myristoyltransferase activity
E	0071222	biological_process	cellular response to lipopolysaccharide
E	0097228	cellular_component	sperm principal piece
E	0106222	molecular_function	lncRNA binding
F	0003723	molecular_function	RNA binding
F	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
F	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
F	0003988	molecular_function	acetyl-CoA C-acyltransferase activity
F	0004300	molecular_function	enoyl-CoA hydratase activity
F	0005515	molecular_function	protein binding
F	0005654	cellular_component	nucleoplasm
F	0005739	cellular_component	mitochondrion
F	0005740	cellular_component	mitochondrial envelope
F	0005741	cellular_component	mitochondrial outer membrane
F	0005743	cellular_component	mitochondrial inner membrane
F	0005783	cellular_component	endoplasmic reticulum
F	0006629	biological_process	lipid metabolic process
F	0006631	biological_process	fatty acid metabolic process
F	0006635	biological_process	fatty acid beta-oxidation
F	0010467	biological_process	gene expression
F	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
F	0016740	molecular_function	transferase activity
F	0016746	molecular_function	acyltransferase activity
F	0016747	molecular_function	acyltransferase activity, transferring groups other than amino-acyl groups
F	0042645	cellular_component	mitochondrial nucleoid
F	0044877	molecular_function	protein-containing complex binding
F	0050633	molecular_function	acetyl-CoA C-myristoyltransferase activity
F	0071222	biological_process	cellular response to lipopolysaccharide
F	0097228	cellular_component	sperm principal piece
F	0106222	molecular_function	lncRNA binding
G	0003824	molecular_function	catalytic activity
G	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
G	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
G	0004300	molecular_function	enoyl-CoA hydratase activity
G	0005515	molecular_function	protein binding
G	0005739	cellular_component	mitochondrion
G	0005743	cellular_component	mitochondrial inner membrane
G	0006629	biological_process	lipid metabolic process
G	0006631	biological_process	fatty acid metabolic process
G	0006635	biological_process	fatty acid beta-oxidation
G	0016491	molecular_function	oxidoreductase activity
G	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
G	0016509	molecular_function	long-chain (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
G	0016616	molecular_function	oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
G	0016740	molecular_function	transferase activity
G	0016829	molecular_function	lyase activity
G	0018812	molecular_function	3-hydroxyacyl-CoA dehydratase activity
G	0032868	biological_process	response to insulin
G	0035965	biological_process	cardiolipin acyl-chain remodeling
G	0042645	cellular_component	mitochondrial nucleoid
G	0052816	molecular_function	long-chain fatty acyl-CoA hydrolase activity
G	0070403	molecular_function	NAD+ binding
H	0003824	molecular_function	catalytic activity
H	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
H	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
H	0004300	molecular_function	enoyl-CoA hydratase activity
H	0005515	molecular_function	protein binding
H	0005739	cellular_component	mitochondrion
H	0005743	cellular_component	mitochondrial inner membrane
H	0006629	biological_process	lipid metabolic process
H	0006631	biological_process	fatty acid metabolic process
H	0006635	biological_process	fatty acid beta-oxidation
H	0016491	molecular_function	oxidoreductase activity
H	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
H	0016509	molecular_function	long-chain (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
H	0016616	molecular_function	oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
H	0016740	molecular_function	transferase activity
H	0016829	molecular_function	lyase activity
H	0018812	molecular_function	3-hydroxyacyl-CoA dehydratase activity
H	0032868	biological_process	response to insulin
H	0035965	biological_process	cardiolipin acyl-chain remodeling
H	0042645	cellular_component	mitochondrial nucleoid
H	0052816	molecular_function	long-chain fatty acyl-CoA hydrolase activity
H	0070403	molecular_function	NAD+ binding
I	0003824	molecular_function	catalytic activity
I	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
I	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
I	0004300	molecular_function	enoyl-CoA hydratase activity
I	0005515	molecular_function	protein binding
I	0005739	cellular_component	mitochondrion
I	0005743	cellular_component	mitochondrial inner membrane
I	0006629	biological_process	lipid metabolic process
I	0006631	biological_process	fatty acid metabolic process
I	0006635	biological_process	fatty acid beta-oxidation
I	0016491	molecular_function	oxidoreductase activity
I	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
I	0016509	molecular_function	long-chain (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
I	0016616	molecular_function	oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
I	0016740	molecular_function	transferase activity
I	0016829	molecular_function	lyase activity
I	0018812	molecular_function	3-hydroxyacyl-CoA dehydratase activity
I	0032868	biological_process	response to insulin
I	0035965	biological_process	cardiolipin acyl-chain remodeling
I	0042645	cellular_component	mitochondrial nucleoid
I	0052816	molecular_function	long-chain fatty acyl-CoA hydrolase activity
I	0070403	molecular_function	NAD+ binding
J	0003824	molecular_function	catalytic activity
J	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
J	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
J	0004300	molecular_function	enoyl-CoA hydratase activity
J	0005515	molecular_function	protein binding
J	0005739	cellular_component	mitochondrion
J	0005743	cellular_component	mitochondrial inner membrane
J	0006629	biological_process	lipid metabolic process
J	0006631	biological_process	fatty acid metabolic process
J	0006635	biological_process	fatty acid beta-oxidation
J	0016491	molecular_function	oxidoreductase activity
J	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
J	0016509	molecular_function	long-chain (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
J	0016616	molecular_function	oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
J	0016740	molecular_function	transferase activity
J	0016829	molecular_function	lyase activity
J	0018812	molecular_function	3-hydroxyacyl-CoA dehydratase activity
J	0032868	biological_process	response to insulin
J	0035965	biological_process	cardiolipin acyl-chain remodeling
J	0042645	cellular_component	mitochondrial nucleoid
J	0052816	molecular_function	long-chain fatty acyl-CoA hydrolase activity
J	0070403	molecular_function	NAD+ binding
K	0003824	molecular_function	catalytic activity
K	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
K	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
K	0004300	molecular_function	enoyl-CoA hydratase activity
K	0005515	molecular_function	protein binding
K	0005739	cellular_component	mitochondrion
K	0005743	cellular_component	mitochondrial inner membrane
K	0006629	biological_process	lipid metabolic process
K	0006631	biological_process	fatty acid metabolic process
K	0006635	biological_process	fatty acid beta-oxidation
K	0016491	molecular_function	oxidoreductase activity
K	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
K	0016509	molecular_function	long-chain (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
K	0016616	molecular_function	oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
K	0016740	molecular_function	transferase activity
K	0016829	molecular_function	lyase activity
K	0018812	molecular_function	3-hydroxyacyl-CoA dehydratase activity
K	0032868	biological_process	response to insulin
K	0035965	biological_process	cardiolipin acyl-chain remodeling
K	0042645	cellular_component	mitochondrial nucleoid
K	0052816	molecular_function	long-chain fatty acyl-CoA hydrolase activity
K	0070403	molecular_function	NAD+ binding
L	0003824	molecular_function	catalytic activity
L	0003857	molecular_function	(3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
L	0003985	molecular_function	acetyl-CoA C-acetyltransferase activity
L	0004300	molecular_function	enoyl-CoA hydratase activity
L	0005515	molecular_function	protein binding
L	0005739	cellular_component	mitochondrion
L	0005743	cellular_component	mitochondrial inner membrane
L	0006629	biological_process	lipid metabolic process
L	0006631	biological_process	fatty acid metabolic process
L	0006635	biological_process	fatty acid beta-oxidation
L	0016491	molecular_function	oxidoreductase activity
L	0016507	cellular_component	mitochondrial fatty acid beta-oxidation multienzyme complex
L	0016509	molecular_function	long-chain (3S)-3-hydroxyacyl-CoA dehydrogenase (NAD+) activity
L	0016616	molecular_function	oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
L	0016740	molecular_function	transferase activity
L	0016829	molecular_function	lyase activity
L	0018812	molecular_function	3-hydroxyacyl-CoA dehydratase activity
L	0032868	biological_process	response to insulin
L	0035965	biological_process	cardiolipin acyl-chain remodeling
L	0042645	cellular_component	mitochondrial nucleoid
L	0052816	molecular_function	long-chain fatty acyl-CoA hydrolase activity
L	0070403	molecular_function	NAD+ binding

Functional Information from PROSITE/UniProt

site_id	PS00067
Number of Residues	25
Details	3HCDH 3-hydroxyacyl-CoA dehydrogenase signature. DgpGFYtTRclaPMMsevir.ILqeG

Chain	Residue	Details
G	ASP541-GLY565

---

site_id	PS00098
Number of Residues	19
Details	THIOLASE_1 Thiolases acyl-enzyme intermediate signature. VTMaCISANqAMttgvglI

Chain	Residue	Details
A	VAL134-ILE152

---

site_id	PS00099
Number of Residues	14
Details	THIOLASE_3 Thiolases active site. GLVAACAAgGqGhA

Chain	Residue	Details
A	GLY453-ALA466

---

site_id	PS00166
Number of Residues	21
Details	ENOYL_COA_HYDRATASE Enoyl-CoA hydratase/isomerase signature. VAaINGsclGGGlevaIsCQY

Chain	Residue	Details
G	VAL138-TYR158

---

site_id	PS00737
Number of Residues	17
Details	THIOLASE_2 Thiolases signature 2. NnwGGsLSlGHPfGaTG

Chain	Residue	Details
A	ASN418-GLY434

---

Functional Information from SwissProt/UniProt

site_id	SWS_FT_FI1
Number of Residues	47
Details	Intramembrane: {"evidences":[{"source":"PubMed","id":"29915090","evidenceCode":"ECO:0000305"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI2
Number of Residues	6
Details	Active site: {"description":"Acyl-thioester intermediate","evidences":[{"source":"PubMed","id":"30850536","evidenceCode":"ECO:0000305"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI3
Number of Residues	6
Details	Active site: {"description":"Proton donor/acceptor","evidences":[{"source":"PubMed","id":"30850536","evidenceCode":"ECO:0000305"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI4
Number of Residues	6
Details	Site: {"description":"Increases nucleophilicity of active site Cys","evidences":[{"source":"PubMed","id":"30850536","evidenceCode":"ECO:0000305"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI5
Number of Residues	19
Details	Modified residue: {"description":"N6-succinyllysine; alternate","evidences":[{"source":"UniProtKB","id":"Q99JY0","evidenceCode":"ECO:0000250"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI6
Number of Residues	13
Details	Modified residue: {"description":"N6-succinyllysine","evidences":[{"source":"UniProtKB","id":"Q99JY0","evidenceCode":"ECO:0000250"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI7
Number of Residues	18
Details	Modified residue: {"description":"N6-acetyllysine","evidences":[{"source":"UniProtKB","id":"Q99JY0","evidenceCode":"ECO:0000250"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI8
Number of Residues	6
Details	Active site: {"description":"For hydroxyacyl-coenzyme A dehydrogenase activity","evidences":[{"source":"PubMed","id":"8770876","evidenceCode":"ECO:0000269"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI9
Number of Residues	12
Details	Site: {"description":"Important for long-chain enoyl-CoA hydratase activity","evidences":[{"source":"PubMed","id":"30850536","evidenceCode":"ECO:0000305"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI10
Number of Residues	6
Details	Site: {"description":"Important for hydroxyacyl-coenzyme A dehydrogenase activity","evidences":[{"source":"PubMed","id":"30850536","evidenceCode":"ECO:0000305"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI11
Number of Residues	114
Details	Modified residue: {"description":"N6-succinyllysine; alternate","evidences":[{"source":"UniProtKB","id":"Q8BMS1","evidenceCode":"ECO:0000250"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI12
Number of Residues	24
Details	Modified residue: {"description":"N6-acetyllysine","evidences":[{"source":"UniProtKB","id":"Q8BMS1","evidenceCode":"ECO:0000250"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI13
Number of Residues	32
Details	Modified residue: {"description":"N6-succinyllysine","evidences":[{"source":"UniProtKB","id":"Q8BMS1","evidenceCode":"ECO:0000250"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI14
Number of Residues	12
Details	Modified residue: {"description":"N6-acetyllysine","evidences":[{"source":"PubMed","id":"19608861","evidenceCode":"ECO:0007744"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI15
Number of Residues	6
Details	Modified residue: {"description":"Phosphoserine","evidences":[{"source":"UniProtKB","id":"Q8BMS1","evidenceCode":"ECO:0000250"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI16
Number of Residues	6
Details	Modified residue: {"description":"Phosphothreonine","evidences":[{"source":"PubMed","id":"24275569","evidenceCode":"ECO:0007744"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI17
Number of Residues	6
Details	Modified residue: {"description":"Omega-N-methylarginine","evidences":[{"source":"UniProtKB","id":"Q8BMS1","evidenceCode":"ECO:0000250"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI18
Number of Residues	12
Details	Modified residue: {"description":"Phosphoserine","evidences":[{"source":"UniProtKB","id":"Q64428","evidenceCode":"ECO:0000250"}]}

Chain

Residue

Details

---

site_id	SWS_FT_FI19
Number of Residues	6
Details	Modified residue: {"description":"Phosphoserine","evidences":[{"source":"PubMed","id":"23186163","evidenceCode":"ECO:0007744"}]}

Chain

Residue

Details

---