{"reference": "J Mol Biol. 2014 Jan 23;426(2):347-61.", "alternative_name": "Ig kappa chain V-I region AU, Ig kappa chain V-I region Ka", "entry": "S-0336", "gene_names": "IGKV1-33", "remarks": "Crystal structure of the amyloid-forming immunoglobulin AL-103 cis-proline 95 mutant", "secondary_structure": "MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK#CCCCCCCCCCCCCCCCCCCCCCCCCEEEECSEEEECTTCCEEEEEEESSCCTTCEEEEEECTTSCCEEEEETTTEECTTCCTTEEEEEETTEEEEEESSCCGGGCEEEEEEECSSSSCCBCCCEEEEEC&MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK#CCCCCCCCCCCCCCCCCCCCCCCCCEEEECSEEEECTTCCEEEEEEESSCCTTCEEEEEECTTSCCEEEEETTTEECTTCCTTEEEEEETTEEEEEESSCCGGGCEEEEEEECSSSSCEECCCEEEEEC&MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK#CCCCCCCCCCCCCCCCCCCCCCCCCEEEECSEEEECTTCCEEEEEEESSCCTTCEEEEEECTTSCCEEEEETTTEECTTCCTTEEEEEETTEEEEEESSCCGGGCEEEEEEECSSSSCCBCCCEEEEEC&MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK#CCCCCCCCCCCCCCCCCCCCCCCCCEEEECSEEEECTTCCEEEEEEESSCCTTCEEEEEECTTSCCEEEEETTTEECTTCCTTEEEEEETTEEEEEESSCCGGGCEEEEEEECSSSSCCBCCCEEEEEC&MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK#CCCCCCCCCCCCCCCCCCCCCCCCCEEEECSEEEECTTCCEEEEEEESSCCTTCEEEEEECTTSCCEEEEETTTEECTTCCTTEEEEEETTEEEEEESSCCGGGCEEEEEEECSSSSCCBCCCEEEEEC&MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK#CCCCCCCCCCCCCCCCCCCCCCCCCEEEECSEEEECTTCCEEEEEEESSCCTTCEEEEEECTTSCCEEEEETTTEECTTCCTTEEEEEETTEEEEEESSCCGGGCEEEEEEECSSSSCCBCCCEEEEEC&MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK#CCCCCCCCCCCCCCCCCCCCCCCCCEEEECSEEEECTTCCEEEEEEESSCCTTCEEEEEECTTSCCEEEEETTTEECTTCCTTEEEEEETTEEEEEESSCCGGGCEEEEEEECSSSSCCBCCCEEEEEC&MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK#CCCCCCCCCCCCCCCCCCCCCCCCCEEEECSEEEECTTCCEEEEEEESSCCTTCEEEEEECTTSCCEEEEETTTEECTTCCTTEEEEEETTEEEEEESSCCGGGCEEEEEEECSSTTCCBCCCEEEEEC&MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK#CCCCCCCCCCCCCCCCCCCCCCCCCEEEECSEEEECTTCCEEEEEEESSCCTTCEEEEEECTTSCCEEEEETTTEECTTCCTTEEEEEETTEEEEEESSCCGGGCEEEEEEECSSSSCCBCCCEEEEEC&MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK#CCCCCCCCCCCCCCCCCCCCCCCCCEEEECSEEEECTTCCEEEEEEESSCCTTCEEEEEECTTSCCEEEEETTTEECTTCCTTEEEEEETTEEEEEESSCCGGGCEEEEEEECSSSSCCBCCCEEEEEC", "chain_id": "B", "inchi": "InChI=1S/H2O4S/c1-5(2,3)4/h(H2,1,2,3,4)/p-2", "amyloid_non_amyloid": "Amyloid", "pmid": "24157440", "ligand_smiles": "[O-]S(=O)(=O)[O-]", "keyword": "Amyloidogenic immunoglobulin light chain protein AL-103", "peptide_protein_sequence": "chain-ID A: MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK; chain-ID B: MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK; chain-ID C: MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK; chain-ID D: MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK; chain-ID E: MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK; chain-ID F: MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK; chain-ID G: MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK; chain-ID H: MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK; chain-ID I: MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK; chain-ID J: MRAKLLGIVLTTPIAISSFASTDIQMTQSPSSLSASVGDRVTITCQASQDISNYLIWYQQKPGKAPKLLIYDASNLETGVPSRFSGSGSGTDFTFTISSLQPEDIATYYCQQYHNLPYTFGPGTKLEIK", "uniprot_ac": "P01594", "protein_name": "Immunoglobulin kappa variable 1-33", "r_value_free": "0.23600000", "mutation_s_field": "[del]P95", "ligand_mw": "96.06", "pdb_id": "4K07", "inchi_key": "QAOWNCQODCNURD-UHFFFAOYSA-L", "ec_number": "", "pdb_classification": "IMMUNE SYSTEM", "type": "Protein", "author": "Blancas-Mejia, L.M., Tischer, A., Thompson, J.R., Tai, J., Wang, L., Auton, M., Ramirez-Alvarado, M.", "species": "Homo sapiens", "ligand_id": "SO4", "method": "X-RAY DIFFRACTION", "ligand_name": "SULFATE ION", "global_stoichiometry": "Homo 2-mer - A2 ", "resolution": "2.83", "ligand_formula": "O4 S -2", "length": "129", "ligstr": "SO4:B:O4 S -2:96.06:SULFATE ION:[O-]S(=O)(=O)[O-]:InChI=1S/H2O4S/c1-5(2,3)4/h(H2,1,2,3,4)/p-2:QAOWNCQODCNURD-UHFFFAOYSA-L", "description": "The deletion of proline 95a (AL-103 delP95aIns), which removes the trans-cis di-proline motif present in the patient protein AL-103, results in a dramatic increment in the thermodynamic stability and a significant delay in fibril formation kinetics with respect to AL-103. Fibril formation is pH dependent; all proteins form fibrils at pH2; reactions become slower and more stochastic as the pH increases up to pH7."}