PMID- 6061750
OWN - NLM
STAT- MEDLINE
DCOM- 19680122
LR  - 20220408
IS  - 0021-9738 (Print)
IS  - 0021-9738 (Linking)
VI  - 46
IP  - 11
DP  - 1967 Nov
TI  - Pathogenesis of hemolytic anemia in homozygous hemoglobin C disease.
PG  - 1795-811
AB  - Hemoglobin C is less soluble than hemoglobin A in red cells, in hemolysates, and 
      in dilute phosphate buffer. Its relative insolubility may be explained by 
      electrostatic interactions between positively charged beta6-lysyl groups and 
      negatively charged groups on adjacent molecules. Red cells from patients with 
      homozygous hemoglobin C (CC) disease exhibit aberrant physical properties which 
      suggest that the cells are more rigid than normal erythrocytes. They pass through 
      membrane filters less readily than normal red cells do, and their viscosity is 
      higher than that of normal cells. Differences from normal cells are exaggerated 
      if mean corpuscular hemoglobin concentration (MCHC) is increased, by suspension 
      in hypertonic salt solution. Increased rigidity of CC cells, by accelerating 
      their fragmentation, may be responsible for formation of microspherocytes. These 
      small dense cells are exceptionally rigid, and probably are even more susceptible 
      to fragmentation and sequestration. Rigidity of CC cells can be attributed to a 
      "precrystalline" state of intracellular hemoglobin, in which crystallization does 
      not occur, although the MCHC exceeds the solubility of hemoglobin in hemolysates.
FAU - Charache, S
AU  - Charache S
FAU - Conley, C L
AU  - Conley CL
FAU - Waugh, D F
AU  - Waugh DF
FAU - Ugoretz, R J
AU  - Ugoretz RJ
FAU - Spurrell, J R
AU  - Spurrell JR
LA  - eng
PT  - Journal Article
PL  - United States
TA  - J Clin Invest
JT  - The Journal of clinical investigation
JID - 7802877
RN  - 9008-00-8 (Hemoglobin C)
SB  - IM
MH  - Anemia, Hemolytic/*pathology
MH  - *Erythrocytes, Abnormal
MH  - Hemoglobin C/*analysis
MH  - Hemoglobin C Disease
MH  - Hemoglobinopathies
MH  - Humans
MH  - Splenomegaly
PMC - PMC292930
EDAT- 1967/11/01 00:00
MHDA- 1967/11/01 00:01
PMCR- 1967/11/01
CRDT- 1967/11/01 00:00
PHST- 1967/11/01 00:00 [pubmed]
PHST- 1967/11/01 00:01 [medline]
PHST- 1967/11/01 00:00 [entrez]
PHST- 1967/11/01 00:00 [pmc-release]
AID - 10.1172/JCI105670 [doi]
PST - ppublish
SO  - J Clin Invest. 1967 Nov;46(11):1795-811. doi: 10.1172/JCI105670.