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1.
Figure 2.

Figure 2. From: Alteration of the unfolded protein response modifies neurodegeneration in a mouse model of Marinesco-Sj?gren syndrome.

A Hyou1transgene confers constitutively elevated expression. (A) Western blot of cerebellar protein extracts from 1-month-old wild-type (+/+), Sil1−/− and Sil1−/−; Tg-Hyou1 mice, probed with an antibody against HYOU1 and an α-tubulin antibody as loading control. (BG) Comparison of HYOU1 expression in Purkinje cells from 1-month-old (B, C), 2-month-old (D, E) Sil1−/− and 1-month-old Sil1−/−; Tg-Hyou1 (F, G) mice. Camera exposure times are equal for images of the same channel. Scale bar = 50 µm.

Lihong Zhao, et al. Hum Mol Genet. 2010 January 1;19(1):25-35.
2.
Figure 5.

Figure 5. From: Alteration of the unfolded protein response modifies neurodegeneration in a mouse model of Marinesco-Sj?gren syndrome.

Hyou1 heterozygosity aggravates ER stress in Sil1−/− Purkinje cells. BiP upregulation in lobule II (AD) or lobule X (EH) of cerebella from 6-week-old Sil1−/− (A, B, E, F) and Sil1−/−; Hyou1+/− mice (C, D, G, H). Sections were subjected to immunostaining with antibodies against BiP and calbindin-D28 (Calb). Camera exposure times are equal for images of the same channel. Scale bar = 100 µm.

Lihong Zhao, et al. Hum Mol Genet. 2010 January 1;19(1):25-35.
3.
Figure 7.

Figure 7. From: Alteration of the unfolded protein response modifies neurodegeneration in a mouse model of Marinesco-Sj?gren syndrome.

Ubiquitin-positive protein inclusions in Purkinje cells. (AI) ER and nuclear localization of protein inclusions. Cerebellar sections from 3-month-old Sil1−/− (A–C), 2-month-old Sil1−/−; Hyou1+/− (D–F) and 3-month-old Sil1−/−; Dnajc3−/− (G–I) mice were immunostained with antibodies against BiP (A, D, G) and ubiquitin (Ub; B, E, H). Merged images are shown in C, F, I. (JL) ERAD chaperone p97/VCP partially co-localizes with ubiquitylated protein inclusions in Sil1−/− Purkinje cells. Brain sections from 80-day-old Sil1−/− mice were subjected to immunohistochemistry with antibodies against p97/VCP (J) and ubiquitin (Ub, K). Merged image is shown in L. Scale bar = 5 µm.

Lihong Zhao, et al. Hum Mol Genet. 2010 January 1;19(1):25-35.
4.
Figure 1.

Figure 1. From: Alteration of the unfolded protein response modifies neurodegeneration in a mouse model of Marinesco-Sj?gren syndrome.

Loss of SIL1 function induces HYOU1 expression in Purkinje cells. Cerebellar sections from 2-month-old wild-type (+/+, A, B, E, F, I, J) or Sil1−/− (C, D, G, H, K, L) mice were immunostained with antibodies to HYOU1 (red) and calbindin-D28 (Calb; green). Images from lobules II (A–D) and lobules X (I–L) are shown. Higher magnification images of A–D are shown in E–H, respectively. Camera exposure times are equal for images of the same channel and magnification. Scale bar = 100 µm.

Lihong Zhao, et al. Hum Mol Genet. 2010 January 1;19(1):25-35.
5.
Figure 4.

Figure 4. From: Alteration of the unfolded protein response modifies neurodegeneration in a mouse model of Marinesco-Sj?gren syndrome.

Deletion of one copy of Hyou1 accelerates Purkinje cell death caused by loss of SIL1 function. (A) Rear stance (gait) on a treadmill test. Mean values ± SEM for the given genotypes and ages are shown. Numbers of mice tested are noted inside the columns for each genotype. *, P < 0.05; **, P < 0.001. (B, C, F, G) Calbindin-D28 immunohistochemistry of 4-week (B), 6-week (C), 8-week (F) or 12-week (G) old Sil1−/−; Hyou1+/− mice. Lobules are indicated by Roman numerals (B). Scale bar = 1 mm. (D, E, H, I). Details of Purkinje cell death in lobule II (D, E, H), and lobule X (I) at ages indicated are shown. Scale bar = 100 µm.

Lihong Zhao, et al. Hum Mol Genet. 2010 January 1;19(1):25-35.
6.
Figure 3.

Figure 3. From: Alteration of the unfolded protein response modifies neurodegeneration in a mouse model of Marinesco-Sj?gren syndrome.

Overexpression of HYOU1 in cerebella prevents Purkinje cell death and ER stress caused by loss of SIL1 function. (AC) Immunohistochemistry using antibody to calbindin-D28 was performed on cerebella from 4-month-old wild-type (A), Sil1−/− (B) and Sil1−/−; Tg-Hyou1 (C) mice. Lobules are indicated by Roman numerals (A). (DI) Expression of Hyou1 transgene suppresses BiP upregulation caused by Sil1 deficiency. Cerebellar sections from 3-month-old wild-type (+/+; D, G), Sil1−/− (E, H) and Sil1−/−; Tg-Hyou1 (F, I) mice were incubated with antibodies against BiP (D–F) and calbindin-D28 (Calb; G–I). Images of lobule II are shown. Camera exposure times are equal for images of the same channel. Scale bars = 1 mm (A–C) and 100 µm (D–I).

Lihong Zhao, et al. Hum Mol Genet. 2010 January 1;19(1):25-35.
7.
Figure 6.

Figure 6. From: Alteration of the unfolded protein response modifies neurodegeneration in a mouse model of Marinesco-Sj?gren syndrome.

Depletion of DNAJC3 activity partially suppresses Purkinje cell death and ER stress caused by SIL1 mutation. (A) Rear stance (gait) on a treadmill test. Mean values ± SEM for the given genotypes at 20 weeks of age are shown. Numbers of mice tested are noted inside the columns for each genotype. *, P < 0.05. (BD) Immunohistochemistry of cerebellar sections from 4-month-old Dnajc3−/− (B), 4-month-old Sil1−/− (C) and 8-month-old Sil1−/−; Dnajc3−/− (D) mice with an antibody against calbindin-D28. Lobules are indicated by Roman numerals (B). (EJ) Expression of BiP (E, G, I) and calbindin-D28 (Calb, F, H, J) in cerebellar Purkinje cells of 3-month-old wild-type (+/+; E, F), Sil1−/− (G, H) and Sil1−/−; Dnajc3−/− (I, J) mice. Camera exposure times are equal for images of the same channel. Scale bars = 1 mm (B–D) and 100 µm (E–J).

Lihong Zhao, et al. Hum Mol Genet. 2010 January 1;19(1):25-35.

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