logo CaMPDB: Calpain for Modulatory Proteolysis Database

SB0040 : [alpha]1-Globin

[ CaMP Format ]

* Basic Information

OrganismHomo sapiens (human)
Protein Nameshemoglobin subunit alpha [Homo sapiens]; hemoglobin subunit alpha; alpha one globin; hemoglobin alpha 1 globin chain; delta globin; alpha-2 globin chain; Hemoglobin subunit alpha; Alpha-globin; Hemoglobin alpha chain
Gene NamesHBA1; HBA2; hemoglobin, alpha 1
Gene Locus16p13.3; chromosome 16
GO FunctionNot available
Entrez Protein Entrez Nucleotide Entrez Gene UniProt OMIM HGNC HPRD KEGG
NP_000549 NM_000558 3039 P69905 141800 HGNC:4823 00784 hsa:3040

* Information From OMIM

Description: The alpha and beta loci determine the structure of the 2 types of polypeptide chains in the tetrameric adult hemoglobin, Hb A, alpha-2/beta-2. The alpha locus also determines a polypeptide chain, the alpha chain, in fetal hemoglobin (alpha-2/gamma-2), in hemoglobin A2(alpha-2/delta-2), and in embryonic hemoglobin (alpha-2/epsilon-2). The number of normal alpha genes (3, 2, 1 or none) in Asian cases of alpha-thalassemia (OMIM:604131) results in 4 different alpha-thalassemia syndromes (Kan et al., 1976). Three normal alpha genes gives a silent carrier state. Two normal alpha genes results in microcytosis (so-called heterozygous alpha-thalassemia). One normal alpha gene results in microcytosis and hemolysis (so-called Hb H disease, OMIM:613978). No normal alpha gene results in 'homozygous alpha-thalassemia' manifested as fatal hydrops fetalis.

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* Structure Information

1. Primary Information

Length: 142 aa

Average Mass: 15.257 kDa

Monoisotopic Mass: 15.248 kDa

2. Domain Information

Annotated Domains: interpro / pfam / smart / prosite

Computationally Assigned Domains (Pfam+HMMER):

domain namebeginendscoree-value
Globin 1. 71071.00.2
--- cleavage 11 (inside Globin 7..107) ---

3. Sequence Information

Fasta Sequence: SB0040.fasta

Amino Acid Sequence and Secondary Structures (PsiPred):

4. 3D Information

Known Structures in PDB: 1A00 (X-ray; 200 A; A/C=2-142), 1A01 (X-ray; 180 A; A/C=2-142), 1A0U (X-ray; 214 A; A/C=2-142), 1A0Z (X-ray; 200 A; A/C=2-142), 1A3N (X-ray; 180 A; A/C=2-142), 1A3O (X-ray; 180 A; A/C=2-142), 1A9W (X-ray; 290 A; A/C=2-142), 1ABW (X-ray; 200 A; A=1-142), 1ABY (X-ray; 260 A; A=1-142), 1AJ9 (X-ray; 220 A; A=2-142), 1B86 (X-ray; 250 A; A/C=2-142), 1BAB (X-ray; 150 A; A/C=1-142), 1BBB (X-ray; 170 A; A/C=2-142), 1BIJ (X-ray; 230 A; A/C=2-142), 1BUW (X-ray; 190 A; A/C=2-142), 1BZ0 (X-ray; 150 A; A/C=2-142), 1BZ1 (X-ray; 159 A; A/C=1-142), 1BZZ (X-ray; 159 A; A/C=2-142), 1C7B (X-ray; 180 A; A/C=2-142), 1C7C (X-ray; 180 A; A=2-142), 1C7D (X-ray; 180 A; A=2-142), 1CLS (X-ray; 190 A; A/C=2-142), 1CMY (X-ray; 300 A; A/C=2-142), 1COH (X-ray; 290 A; A/C=2-142), 1DKE (X-ray; 210 A; A/C=2-142), 1DXT (X-ray; 170 A; A/C=2-142), 1DXU (X-ray; 170 A; A/C=2-142), 1DXV (X-ray; 170 A; A/C=2-142), 1FDH (X-ray; 250 A; A/B=2-142), 1FN3 (X-ray; 248 A; A/C=2-142), 1G9V (X-ray; 185 A; A/C=2-142), 1GBU (X-ray; 180 A; A/C=2-142), 1GBV (X-ray; 200 A; A/C=2-142), 1GLI (X-ray; 250 A; A/C=3-142), 1GZX (X-ray; 210 A; A/C=2-142), 1HAB (X-ray; 230 A; A/C=2-142), 1HAC (X-ray; 260 A; A/C=2-142), 1HBA (X-ray; 210 A; A/C=2-142), 1HBB (X-ray; 190 A; A/C=2-142), 1HBS (X-ray; 300 A; A/C/E/G=2-142), 1HCO (X-ray; 270 A; A=2-142), 1HDB (X-ray; 220 A; A/C=2-142), 1HGA (X-ray; 210 A; A/C=2-142), 1HGB (X-ray; 210 A; A/C=2-142), 1HGC (X-ray; 210 A; A/C=2-142), 1HHO (X-ray; 210 A; A=2-142), 1IRD (X-ray; 125 A; A=2-142), 1J3Y (X-ray; 155 A; A/C/E/G=2-142), 1J3Z (X-ray; 160 A; A/C/E/G=2-142), 1J40 (X-ray; 145 A; A/C/E/G=2-142), 1J41 (X-ray; 145 A; A/C/E/G=2-142), 1J7S (X-ray; 220 A; A/C=2-142), 1J7W (X-ray; 200 A; A/C=2-142), 1J7Y (X-ray; 170 A; A/C=2-142), 1JY7 (X-ray; 320 A; A/C/P/R/U/W=2-142), 1K0Y (X-ray; 187 A; A/C=2-142), 1K1K (X-ray; 200 A; A=2-142), 1KD2 (X-ray; 187 A; A/C=2-142), 1LFL (X-ray; 270 A; A/C/P/R=2-142), 1LFQ (X-ray; 260 A; A=2-142), 1LFT (X-ray; 260 A; A=2-142), 1LFV (X-ray; 280 A; A=2-142), 1LFY (X-ray; 330 A; A=2-142), 1LFZ (X-ray; 310 A; A=2-142), 1LJW (X-ray; 216 A; A=2-142), 1M9P (X-ray; 210 A; A/C=2-142), 1MKO (X-ray; 218 A; A/C=2-142), 1NEJ (X-ray; 210 A; A/C=2-142), 1NIH (X-ray; 260 A; A/C=2-142), 1NQP (X-ray; 173 A; A/C=2-142), 1O1I (X-ray; 230 A; A=2-142), 1O1J (X-ray; 190 A; A=2-142), 1O1K (X-ray; 200 A; A/C=3-142), 1O1L (X-ray; 180 A; A=2-142), 1O1M (X-ray; 185 A; A=2-142), 1O1N (X-ray; 180 A; A=2-142), 1O1O (X-ray; 180 A; A/C=2-142), 1O1P (X-ray; 180 A; A=2-142), 1QI8 (X-ray; 180 A; A/C=3-142), 1QSH (X-ray; 170 A; A/C=2-142), 1QSI (X-ray; 170 A; A/C=2-142), 1QXD (X-ray; 225 A; A/C=2-142), 1QXE (X-ray; 185 A; A/C=2-142), 1R1X (X-ray; 215 A; A=2-142), 1R1Y (X-ray; 180 A; A/C=2-142), 1RPS (X-ray; 211 A; A/C=2-142), 1RQ3 (X-ray; 191 A; A/C=2-142), 1RQ4 (X-ray; 211 A; A/C=2-142), 1RQA (X-ray; 211 A; A/C=2-142), 1RVW (X-ray; 250 A; A=2-142), 1SDK (X-ray; 180 A; A/C=2-142), 1SDL (X-ray; 180 A; A/C=2-142), 1SHR (X-ray; 188 A; A/C=2-142), 1SI4 (X-ray; 220 A; A/C=2-142), 1THB (X-ray; 150 A; A/C=2-142), 1UIW (X-ray; 150 A; A/C/E/G=2-142), 1VWT (X-ray; 190 A; A/C=2-142), 1XXT (X-ray; 191 A; A/C=2-142), 1XY0 (X-ray; 199 A; A/C=2-142), 1XYE (X-ray; 213 A; A/C=3-142), 1XZ2 (X-ray; 190 A; A/C=2-142), 1XZ4 (X-ray; 200 A; A/C=3-142), 1XZ5 (X-ray; 211 A; A/C=2-142), 1XZ7 (X-ray; 190 A; A/C=2-142), 1XZU (X-ray; 216 A; A/C=2-142), 1XZV (X-ray; 211 A; A/C=2-142), 1Y01 (X-ray; 280 A; B=1-142), 1Y09 (X-ray; 225 A; A/C=2-142), 1Y0A (X-ray; 222 A; A/C=2-140), 1Y0C (X-ray; 230 A; A/C=2-140), 1Y0D (X-ray; 210 A; A/C=2-141), 1Y0T (X-ray; 214 A; A/C=2-142), 1Y0W (X-ray; 214 A; A/C=2-142), 1Y22 (X-ray; 216 A; A/C=2-142), 1Y2Z (X-ray; 207 A; A/C=2-142), 1Y31 (X-ray; 213 A; A/C=2-142), 1Y35 (X-ray; 212 A; A/C=2-142), 1Y45 (X-ray; 200 A; A/C=2-142), 1Y46 (X-ray; 222 A; A/C=2-142), 1Y4B (X-ray; 210 A; A/C=2-142), 1Y4F (X-ray; 200 A; A/C=2-142), 1Y4G (X-ray; 191 A; A/C=2-142), 1Y4P (X-ray; 198 A; A/C=2-142), 1Y4Q (X-ray; 211 A; A/C=2-142), 1Y4R (X-ray; 222 A; A/C=2-142), 1Y4V (X-ray; 184 A; A/C=2-142), 1Y5F (X-ray; 214 A; A/C=2-142), 1Y5J (X-ray; 203 A; A/C=2-142), 1Y5K (X-ray; 220 A; A/C=2-142), 1Y7C (X-ray; 210 A; A/C=2-142), 1Y7D (X-ray; 190 A; A/C=2-142), 1Y7G (X-ray; 210 A; A/C=2-142), 1Y7Z (X-ray; 198 A; A/C=2-142), 1Y83 (X-ray; 190 A; A/C=2-142), 1Y85 (X-ray; 213 A; A/C=2-142), 1Y8W (X-ray; 290 A; A/C=2-142), 1YDZ (X-ray; 330 A; A/C=2-140), 1YE0 (X-ray; 250 A; A/C=2-142), 1YE1 (X-ray; 450 A; A/C=2-142), 1YE2 (X-ray; 180 A; A/C=2-142), 1YEN (X-ray; 280 A; A/C=2-142), 1YEO (X-ray; 222 A; A/C=2-142), 1YEQ (X-ray; 275 A; A/C=2-142), 1YEU (X-ray; 212 A; A/C=2-142), 1YEV (X-ray; 211 A; A/C=2-142), 1YFF (X-ray; 240 A; A/C/E/G=2-142), 1YG5 (X-ray; 270 A; A/C=2-142), 1YGD (X-ray; 273 A; A/C=2-142), 1YGF (X-ray; 270 A; A/C=2-142), 1YH9 (X-ray; 220 A; A/C=2-142), 1YHE (X-ray; 210 A; A/C=2-142), 1YHR (X-ray; 260 A; A/C=2-142), 1YIE (X-ray; 240 A; A/C=2-142), 1YIH (X-ray; 200 A; A/C=2-142), 1YVQ (X-ray; 180 A; A/C=2-142), 1YVT (X-ray; 180 A; A=2-142), 1YZI (X-ray; 207 A; A=2-142), 1Z8U (X-ray; 240 A; B/D=1-142), 2D5Z (X-ray; 145 A; A/C=2-142), 2D60 (X-ray; 170 A; A/C=2-142), 2DN1 (X-ray; 125 A; A=2-142), 2DN2 (X-ray; 125 A; A/C=2-142), 2DN3 (X-ray; 125 A; A=2-142), 2DXM (Neutron; 210 A; A/C=2-142), 2H35 (NMR; -; A/C=2-142), 2HBC (X-ray; 210 A; A=2-142), 2HBD (X-ray; 220 A; A=2-142), 2HBE (X-ray; 200 A; A=2-142), 2HBF (X-ray; 220 A; A=2-142), 2HBS (X-ray; 205 A; A/C/E/G=2-142), 2HCO (X-ray; 270 A; A=2-142), 2HHB (X-ray; 174 A; A/C=2-142), 2HHD (X-ray; 220 A; A/C=2-142), 2HHE (X-ray; 220 A; A/C=2-142), 2M6Z (NMR; -; A/C=2-142), 2W6V (X-ray; 180 A; A/C=2-142), 2W72 (X-ray; 107 A; A=2-142, C=3-142), 2YRS (X-ray; 230 A; A/C/I/M=2-142), 3B75 (X-ray; 230 A; A/C/E/G/S=2-142), 3D17 (X-ray; 280 A; A/C=2-142), 3D7O (X-ray; 180 A; A=2-142), 3DUT (X-ray; 155 A; A/C=2-142), 3HHB (X-ray; 174 A; A/C=2-142), 3HXN (X-ray; 200 A; A/C=2-142), 3IA3 (X-ray; 320 A; B/D=1-142), 3IC0 (X-ray; 180 A; A/C=2-142), 3IC2 (X-ray; 240 A; A/C=2-142), 3KMF (Neutron; 200 A; A/E=2-142), 3NL7 (X-ray; 180 A; A=2-142), 3NMM (X-ray; 160 A; A/C=2-142), 3ODQ (X-ray; 310 A; A/C=2-142), 3ONZ (X-ray; 209 A; A=2-142), 3OO4 (X-ray; 190 A; A=2-142), 3OO5 (X-ray; 210 A; A=2-142), 3OVU (X-ray; 283 A; C=2-142), 3P5Q (X-ray; 200 A; A=2-142), 3QJB (X-ray; 180 A; A=2-142), 3QJC (X-ray; 200 A; A=2-142), 3QJD (X-ray; 156 A; A/C=2-142), 3QJE (X-ray; 180 A; A/C=2-142), 3R5I (X-ray; 220 A; A/C=2-142), 3S48 (X-ray; 305 A; C/D=2-142), 3S65 (X-ray; 180 A; A/C=2-142), 3S66 (X-ray; 140 A; A=2-142), 3SZK (X-ray; 301 A; A/D=2-142), 3WCP (X-ray; 194 A; A/C=2-142), 3WHM (X-ray; 185 A; A/E=2-142), 4FC3 (X-ray; 226 A; A=2-142), 4HHB (X-ray; 174 A; A/C=2-142), 4IJ2 (X-ray; 424 A; A/C=2-142), 4L7Y (X-ray; 180 A; A/C=2-142), 4M4A (X-ray; 205 A; A=2-142), 4M4B (X-ray; 200 A; A=2-142), 4MQC (X-ray; 220 A; A=2-142), 4MQG (X-ray; 168 A; A=2-142), 4MQH (X-ray; 250 A; A=2-140), 4MQI (X-ray; 192 A; A=2-141), 4MQJ (X-ray; 180 A; A/C/E/G=2-142), 4MQK (X-ray; 224 A; A/C/E/G=2-142), 4N7N (X-ray; 275 A; A/C/E/G/I/K=2-142), 4N7O (X-ray; 250 A; A/C/E/G/I/K=2-142), 4N7P (X-ray; 281 A; A/C/E/G/I/K=2-142), 4N8T (X-ray; 190 A; A=2-142), 4NI0 (X-ray; 215 A; A=2-142), 4NI1 (X-ray; 190 A; A=2-142), 4WJG (X-ray; 310 A; A/F/K/P/U/Z=2-142), 4X0I (X-ray; 309 A; A=2-142), 4X0L (X-ray; 205 A; A=2-142), 6HBW (X-ray; 200 A; A/C=2-142)

* Cleavage Information

1 [sites] cleaved by Calpain 1

Source Reference: [PubMed ID: 6331510] Melloni E, Salamino F, Sparatore B, Michetti M, Pontremoli S, Characterization of the single peptide generated from the amino-terminus end of alpha- and beta-hemoglobin chains by the Ca2+-dependent neutral proteinase. Biochim Biophys Acta. 1984 Jul 17;788(1):11-6.

Cleavage sites (±10aa)

[Site 1] VLSPADKTNV11-KAAWGKVGAH

Val11 Lys

P10 P9 P8 P7 P6 P5 P4 P3 P2 P1
Val2Leu3Ser4Pro5Ala6Asp7Lys8Thr9Asn10Val11
P1' P2' P3' P4' P5' P6' P7' P8' P9' P10'
Lys12Ala13Ala14Trp15Gly16Lys17Val18Gly19Ala20His21

Sequence conservation (by blast)

* References

[PubMed ID: 25284125] Farra C, Badra R, Fares F, Muwakkit S, Dbaibo G, Dabbous I, Ashkar H, Mounsef C, Abboud MR, Alpha thalassemia allelic frequency in Lebanon. Pediatr Blood Cancer. 2015 Jan;62(1):120-2. doi: 10.1002/pbc.25242. Epub 2014 Oct

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