1DE4: Hemochromatosis protein hfe

Hemochromatosis protein hfe complexed with transferrin receptor. Determined by X-ray diffraction at 2.8 Å resolution. Released 19 Jan 2000.

Method: X-ray diffraction
Resolution: 2.8 Å
Organism: Homo sapiens
Chains: 9
Atoms: 24,315
Mol. weight: 348.56 kDa
Ligands: NAG, CA
Released: 19 Jan 2000

Explore 1DE4 in 3D Show helices and sheets RCSB PDB PDBe

Secondary structure: helices and β-sheets

1DE4 contains 123 α-helices and 171 β-strands across 9 chains. Residue ranges use author residue numbering, as in the PDB file, from PDBe. To see them in 3D, choose the Cartoon representation with Secondary structure coloring: helices, sheets and coils get different colors.

Chains A and G: 11 helices, 18 β-strands

Element	Residues	Length	Sheet
β-strand	6-15	10	1
β-strand	25-31	7	1
β-strand	34-39	6	1
β-strand	46-48	3	1
α-helix	53-56	4
α-helix	59-85	27
β-strand	95-104	10	1
β-strand	110-118	9	1
β-strand	121-127	7	1
α-helix	128-130	3
β-strand	132-135	4	1
α-helix	138-140	3
α-helix	141-150	10
α-helix	152-159	8
α-helix	160-164	5
α-helix	165-174	10
β-strand	184	1	2
β-strand	187-194	8	3
β-strand	199-208	10	3
β-strand	209	1	2
β-strand	214-219	6	4
β-strand	222-223	2	4
α-helix	226-228	3
α-helix	230-232	3
β-strand	233-236	4	3
β-strand	242-250	9	3
α-helix	255-257	3
β-strand	258-263	6	4
β-strand	271-273	3	4

Chains B, E and H: 0 helices, 10 β-strands

Element	Residues	Length	Sheet
β-strand	6	1	5
β-strand	9-11	3	5
β-strand	21-30	10	5
β-strand	36-41	6	6
β-strand	44-45	2	6
β-strand	50-51	2	5
β-strand	55-56	2	5
β-strand	62-70	9	5
β-strand	78-83	6	6
β-strand	91-94	4	6

Chain C: 31 helices, 29 β-strands

Element	Residues	Length	Sheet
α-helix	124-136	13
α-helix	140-146	7
α-helix	160-175	16
β-strand	180-192	13	7
α-helix	197-198	2
β-strand	199-204	6	8
β-strand	209-210	2	8
β-strand	214	1	8
β-strand	220-221	2	9
α-helix	223-225	3
β-strand	226-230	5	8
β-strand	232-234	3	10
α-helix	240-244	5
β-strand	254-258	5	10
β-strand	260	1	11
β-strand	262	1	11
α-helix	264-273	10
β-strand	279-282	4	10
β-strand	299-300	2	9
α-helix	317-319	3
β-strand	334-336	3	10
α-helix	339-346	8
β-strand	349	1	12
β-strand	352	1	13
α-helix	353-354	2
α-helix	355-357	3
β-strand	364	1	13
β-strand	367	1	12
β-strand	371-377	7	8
β-strand	380-393	14	7
β-strand	398-408	11	7
α-helix	416-420	5
α-helix	421-438	18
β-strand	446-453	8	7
α-helix	461-469	9
α-helix	471-474	4
β-strand	478-483	6	7
β-strand	488	1	7
β-strand	493-498	6	7
α-helix	500-502	3
α-helix	503-512	10
β-strand	514	1	14
β-strand	521	1	14
α-helix	528-531	4
α-helix	532-535	4
α-helix	541-543	3
α-helix	544-548	5
β-strand	552-558	7	7
α-helix	573-579	7
α-helix	583-602	20
α-helix	611-613	3
α-helix	614-626	13
α-helix	629-634	6
α-helix	640-661	22
α-helix	670-683	14
β-strand	686	1	15
β-strand	699	1	15
α-helix	709-718	10
α-helix	728-749	22
α-helix	753-755	3

Chain D: 11 helices, 18 β-strands

Element	Residues	Length	Sheet
β-strand	6-15	10	16
β-strand	25-31	7	16
β-strand	34-39	6	16
β-strand	46-48	3	16
α-helix	53-56	4
α-helix	59-85	27
β-strand	95-104	10	16
β-strand	110-118	9	16
β-strand	121-127	7	16
α-helix	128-130	3
β-strand	132-135	4	16
α-helix	138-140	3
α-helix	141-150	10
α-helix	152-159	8
α-helix	160-164	5
α-helix	165-174	10
β-strand	184	1	17
β-strand	187-194	8	18
β-strand	199-208	10	18
β-strand	209	1	17
β-strand	214-219	6	19
β-strand	222-223	2	19
α-helix	226-228	3
α-helix	230-232	3
β-strand	233-237	5	18
β-strand	241-250	10	18
α-helix	255-257	3
β-strand	258-263	6	19
β-strand	271-273	3	19

Chain F: 30 helices, 29 β-strands

Element	Residues	Length	Sheet
α-helix	124-136	13
α-helix	140-146	7
α-helix	160-175	16
β-strand	180-192	13	23
α-helix	197-198	2
β-strand	199-204	6	24
β-strand	208-209	2	24
β-strand	214	1	24
β-strand	219-221	3	25
β-strand	226-230	5	24
β-strand	232-234	3	26
α-helix	240-244	5
β-strand	254-258	5	26
β-strand	260	1	27
β-strand	262	1	27
α-helix	264-273	10
β-strand	279-282	4	26
β-strand	298-300	3	25
α-helix	317-319	3
β-strand	334-336	3	26
α-helix	339-346	8
β-strand	349	1	28
β-strand	352	1	29
α-helix	353-354	2
α-helix	355-357	3
β-strand	364	1	29
β-strand	367	1	28
β-strand	371-377	7	24
β-strand	380-393	14	23
β-strand	398-408	11	23
α-helix	416-420	5
α-helix	421-438	18
β-strand	446-453	8	23
α-helix	461-469	9
α-helix	471-474	4
β-strand	478-483	6	23
β-strand	488	1	23
β-strand	493-498	6	23
α-helix	500-502	3
α-helix	503-512	10
β-strand	514	1	30
β-strand	521	1	30
α-helix	528-531	4
α-helix	532-535	4
α-helix	541-543	3
α-helix	544-548	5
β-strand	552-558	7	23
α-helix	573-579	7
α-helix	583-602	20
α-helix	611-613	3
α-helix	614-626	13
α-helix	629-634	6
α-helix	640-661	22
α-helix	670-683	14
β-strand	686	1	31
β-strand	699	1	31
α-helix	709-718	10
α-helix	728-749	22
α-helix	753-755	3

Chain I: 29 helices, 29 β-strands

Element	Residues	Length	Sheet
α-helix	124-136	13
α-helix	140-146	7
α-helix	160-175	16
β-strand	180-192	13	39
α-helix	197-198	2
β-strand	199-204	6	40
β-strand	208-209	2	40
β-strand	214	1	40
β-strand	220-221	2	41
β-strand	226-230	5	40
β-strand	232-234	3	42
α-helix	240-244	5
β-strand	254-258	5	42
β-strand	260	1	43
β-strand	262	1	43
α-helix	264-272	9
β-strand	279-282	4	42
β-strand	299-300	2	41
α-helix	317-319	3
β-strand	334-336	3	42
α-helix	339-346	8
β-strand	349	1	44
β-strand	352	1	45
α-helix	353-354	2
α-helix	355-357	3
β-strand	364	1	45
β-strand	367	1	44
β-strand	371-377	7	40
β-strand	380-393	14	39
β-strand	398-408	11	39
α-helix	416-420	5
α-helix	421-438	18
β-strand	446-453	8	39
α-helix	461-469	9
α-helix	471-474	4
β-strand	478-483	6	39
β-strand	488	1	39
β-strand	493-498	6	39
α-helix	500-502	3
α-helix	503-512	10
β-strand	514	1	46
β-strand	521	1	46
α-helix	528-531	4
α-helix	532-535	4
α-helix	541-543	3
α-helix	544-548	5
β-strand	552-558	7	39
α-helix	573-579	7
α-helix	583-602	20
α-helix	613-626	14
α-helix	629-634	6
α-helix	640-661	22
α-helix	670-683	14
β-strand	686	1	47
β-strand	699	1	47
α-helix	709-718	10
α-helix	728-749	22
α-helix	753-755	3

Molecules and chains

Molecule	Chains	Type	Length	Organism	UniProt
Hemochromatosis protein	A, D, G	protein	275	Homo sapiens	Q30201 (AlphaFold model)
Beta-2-microglobulin	B, E, H	protein	99	Homo sapiens	P61769 (AlphaFold model)
Transferrin receptor	C, F, I	protein	640	Homo sapiens	P02786 (AlphaFold model)

Sequence of entity 1 (A, D, G), FASTA

>1DE4_1 HEMOCHROMATOSIS PROTEIN (chains A, D, G)
RLLRSHSLHYLFMGASEQDLGLSLFEALGYVDDQLFVFYDHESRRVEPRTPWVSSRISSQ
MWLQLSQSLKGWDHMFTVDFWTIMENHNHSKESHTLQVILGCEMQEDNSTEGYWKYGYDG
QDHLEFCPDTLDWRAAEPRAWPTKLEWERHKIRARQNRAYLERDCPAQLQQLLELGRGVL
DQQVPPLVKVTHHVTSSVTTLRCRALNYYPQNITMKWLKDKQPMDAKEFEPKDVLPNGDG
TYQGWITLAVPPGEEQRYTCQVEHPGLDQPLIVIW

Sequence of entity 2 (B, E, H), FASTA

>1DE4_2 BETA-2-MICROGLOBULIN (chains B, E, H)
IQRTPKIQVYSRHPAENGKSNFLNCYVSGFHPSDIEVDLLKNGERIEKVEHSDLSFSKDW
SFYLLYYTEFTPTEKDEYACRVNHVTLSQPKIVKWDRDM

Sequence of entity 3 (C, F, I), FASTA

>1DE4_3 TRANSFERRIN RECEPTOR (chains C, F, I)
RLYWDDLKRKLSEKLDSTDFTSTIKLLNENSYVPREAGSQKDENLALYVENQFREFKLSK
VWRDQHFVKIQVKDSAQNSVIIVDKNGRLVYLVENPGGYVAYSKAATVTGKLVHANFGTK
KDFEDLYTPVNGSIVIVRAGKITFAEKVANAESLNAIGVLIYMDQTKFPIVNAELSFFGH
AHLGTGDPYTPGFPSFNHTQFPPSRSSGLPNIPVQTISRAAAEKLFGNMEGDCPSDWKTD
STCRMVTSESKNVKLTVSNVLKEIKILNIFGVIKGFVEPDHYVVVGAQRDAWGPGAAKSG
VGTALLLKLAQMFSDMVLKDGFQPSRSIIFASWSAGDFGSVGATEWLEGYLSSLHLKAFT
YINLDKAVLGTSNFKVSASPLLYTLIEKTMQNVKHPVTGQFLYQDSNWASKVEKLTLDNA
AFPFLAYSGIPAVSFCFCEDTDYPYLGTTMDTYKELIERIPELNKVARAAAEVAGQFVIK
LTHDVELNLDYERYNSQLLSFVRDLNQYRADIKEMGLSLQWLYSARGDFFRATSRLTTDF
GNAEKTDRFVMKKLNDRVMRVEYHFLSPYVSPKESPFRHVFWGSGSHTLPALLENLKLRK
QNNGAFNETLFRNQLALATWTIQGAANALSGDVWDIDNEF

Ligands and cofactors

ID	Name	Formula	Copies
NAG	2-acetamido-2-deoxy-beta-D-glucopyranose	C8 H15 N O6	3
CA	Calcium ion	Ca	3

Water and common crystallization additives (GOL) are not listed.

Primary citation

Crystal structure of the hereditary haemochromatosis protein HFE complexed with transferrin receptor. Bennett, M.J., Lebron, J.A., Bjorkman, P.J. Nature (2000) 403:46-53. DOI 10.1038/47417 · PubMed

Other PDB entries of the same protein (UniProt Q30201 (AlphaFold model), which also has an AlphaFold model), best resolution first:

1A6Z 2.6 Å, Hfe (HUMAN) hemochromatosis protein

Browse structure collections

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