https://orcid.org/0000-0001-8206-2288
Figures & data
Table 1 Summary and comparison between clinical features, biochemical analysis and gene analysis in six patients with HS.
Figure 1. (HS1–HS3) . (HS4–HS6). Erythrocyte membrane proteins in six patients with hereditary spherocytosis . (A) Eosin-5′-maleimide (EMA) staining (B) fluorescein isothiocyanate (FITC) staining of the intracellular domain of band 3. (C) Levels of band 3 protein in red blood cell membranes as determined by SDS-PAGE and Coomassie staining. (D) Densitometrical wave (E) MAS: mass spectrometry (spectrum patterns).
Figure 2. (HS1–HS3) . (HS4–HS6). Erythrocyte membrane proteins in six patients with hereditary spherocytosis. (A) Eosin-5′-maleimide (EMA) staining (B) fluorescein isothiocyanate (FITC) staining of the intracellular domain of band 3. (C) Levels of band 3 protein in red blood cell membranes as determined by SDS-PAGE and Coomassie staining. (D) Densitometrical wave (E) MAS: mass spectrometry (spectrum patterns).
Figure 3. Western blotting (anti-spectrin Ab, anti-spectrin Ab, anti-band 3 Ab, anti-actin Ab).
Figure 4. Mechanism of low-molecular-weight spectrin, and ankyrin production and loss of these protein complexes from the erythrocyte membrane due to specific gene anomalies observed in six patients with hereditary spherocytosis (HS1–HS6). HS1: Piece of band 3 released from the membrane because of a conformational change in intracellular band 3 during circulation in the spleen. HS2: Stop codon appears at 10 (distal area), and one allele missing seven segments forms the binding site and causes a lack of binding protein. HS3: Stop codon appears at 10 (distal area), and one allele missing seven segments forms the binding site and causes a lack of binding protein. HS4: Stop codon appears at 5 (proximal area) in the DNA sequence, and one allele missing 12 segments forms the binding site and causes a lack of binding protein. HS5: Stop codon appears at 10 (distal area) in the DNA sequence, and one allele missing 14 segments, comprising band 3 and spectrin, causes a lack of protein binding to half of band 3 and all spectrin. HS6: Stop codon appears at 3 (proximal area) in the DNA sequence, and one allele missing 21 segments, which comprises band 3 and spectrin, causes a lack of protein binding to all band 3 and spectrin.
