Hemoglobin Rouen (alpha-140 (HC2) Tyr-->His): alteration of the alpha-chain C-terminal region and moderate increase in oxygen affinity.
Hb Rouen (alpha 140(HC2) Tyr-->His) is a moderately high oxygen-affinity variant that was found in coincidence with polycythemia vera in a French patient. This hemoglobin provides an example of an alteration of the C-terminus of the alpha-chain, a region involved in the mechanisms of allosteric regulation. The increase in oxygen-affinity and decrease in cooperativity of this variant is much smaller than that resulting from the same substitution in the beta-chain. This model provides additional evidence for the inequivalence between the alpha- and beta-subunits.
Wajcman
H
H
INSERM U299, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France.
Kister
J
J
Marden
M
M
Lahary
A
A
Monconduit
M
M
Galacteros
F
F
D000368:Aged; D000369:Aged, 80 and over; D000494:Allosteric Regulation; D000596:Amino Acids / Q000032:analysis; D002248:Carbon Monoxide / Q000378:metabolism; D002851:Chromatography, High Pressure Liquid; D006455:Hemoglobins, Abnormal / Q000737:chemistry / Q000235:genetics* / Q000378:metabolism; D006639:Histidine / Q000235:genetics; D006801:Humans; D007700:Kinetics; D008297:Male; D009154:Mutation; D010100:Oxygen / Q000378:metabolism*; D011087:Polycythemia Vera / Q000097:blood; D012697:Serine Endopeptidases / Q000737:chemistry; D014443:Tyrosine / Q000235:genetics
D000596:Amino Acids; D006455:Hemoglobins, Abnormal; C077415:hemoglobin Rouen; D014443:Tyrosine; D006639:Histidine; D002248:Carbon Monoxide; D012697:Serine Endopeptidases; C028344:glutamyl endopeptidase; D010100:Oxygen