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Blood, Vol. 108, Issue 2, 493-500, July 15, 2006
Mutations in neutrophil elastase causing congenital neutropenia lead to cytoplasmic protein accumulation and induction of the unfolded protein response
Blood Köllner et al.
108: 493
Suppemental materials for: Köllner
Files in this Data Supplement:
- Figure S1. Subcellular localization of WT-HNE protein in transiently transfected HeLa cells (JPG, 68.7 KB)
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Cells were labelled with anti-HNE (green) and anti-calnexin (ER marker; red), anti-giantin (Golgi marker; red), and anti-lamp-3 (lysosomal marker; red). TO-PRO-3 was used to counterstain the nuclei (blue). Yellow displays colocalization of HNE protein with organelle-specific markers.
- Figure S2. Subcellular localization of MT-HNE protein (C122Y) in transiently transfected HeLa cells (JPG, 76.8 KB)
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Cells were labelled with anti-HNE (green) and anti-calnexin (ER marker; red), anti-giantin (Golgi marker; red), and anti-lamp-3 (lysosomal marker; red). TO-PRO-3 was used to counterstain the nuclei (blue). Yellow displays colocalization of HNE protein with organelle-specific markers.
- Figure S3. Subcellular localization of MT-HNE protein (ΔV161-F170) in transiently transfected HeLa cells (JPG, 78.4 KB)
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Cells were labelled with anti-HNE (green) and anti-calnexin (ER marker; red), anti-giantin (Golgi marker; red), and anti-lamp-3 (lysosomal marker; red). TO-PRO-3 was used to counterstain the nuclei (blue). Yellow displays colocalization of HNE protein with organelle-specific markers.
- Figure S4. Subcellular localization of MT-HNE protein (P176R) in transiently transfected HeLa cells (JPG, 76.5 KB)
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Cells were labelled with anti-HNE (green) and anti-calnexin (ER marker; red), anti-giantin (Golgi marker; red), and anti-lamp-3 (lysosomal marker; red). TO-PRO-3 was used to counterstain the nuclei (blue). Yellow displays colocalization of HNE protein with organelle-specific markers.
- Figure S5. Subcellular localization of MT-HNE protein (R191Q) in transiently transfected HeLa cells (JPG, 63.8 KB)
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Cells were labelled with anti-HNE (green) and anti-calnexin (ER marker; red), anti-giantin (Golgi marker; red), and anti-lamp-3 (lysosomal marker; red). TO-PRO-3 was used to counterstain the nuclei (blue). Yellow displays colocalization of HNE protein with organelle-specific markers.
- Figure S6. Intracellular mistrafficking and cytoplasmic accumulation of MT-HNE proteins in neutrophil granulocytes of patients suffering from SCN (JPG, 92 KB)
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Subcellular localization of WT-HNE in granulocytes of healthy donors with or without rh-G-CSF treatment, or of MT-HNE in granulocytes of patients suffering from SCN and carrying heterozygous mutations in ELA2 (pat #1: C122Y; pat #2:  V161-F170). Cells were stained with anti-HNE (green), and anti-lamp-3 (lysosomal marker; red), or only anti-MPO (azurophil granules marker; red). TO-PRO-3 was used to counterstain the nuclei (blue). Yellow represents colocalization of HNE protein with lamp-3.
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