ZFIN ID: ZDB-FIG-140530-8
Blanco et al., 2014 - Complexes of Usher proteins preassemble at the endoplasmic reticulum and are required for trafficking and ER homeostasis. Disease models & mechanisms   7:547-59 Full text @ Dis. Model. Mech.
ADDITIONAL FIGURES
EXPRESSION / LABELING:
Genes: adgrv1, whrnb
Antibody: Ab1-ush2a
Fish: WT, cdh23tj264/tj264, ift88tz288/tz288, myo7aaty220/ty220, ush1cfh293/fh293
Anatomical Term: hair cell
Stage: Day 5

Fig. 7 The Cdh23, Harmonin and Myo7aa protein complex is required for proper trafficking of USH2 proteins and can trigger ER stress when defective. (A–O) Immunolabeling of USH2 proteins in (A–C) wild-type (WT) siblings, and (D–F) cdh23, (G–I) ush1c, (J–L) ift88 and (M–O) myo7aa mutants with antibodies against Ush2a (A,D,G,J,M), Gpr98 (B,E,H,K,N) and Whirlinb (C,F,I,L,O). (P) Levels of hspa5 expression based on in situ hybridization technique in whole-mount larvae. For ush1c siblings n=57; ush1c/ n=51; cdh23 siblings n=35; for cdh23/ n=33; myo7aa siblings n=24; for myo7aa/ n=24 analyzed anterior maculae. (Q) Quantification of percentage area per hair cell containing KDEL expression. For ush1c siblings n=85; ush1c/ n=125; cdh23 siblings n=66; for cdh23/ n=60; myo7aa siblings n=60; myo7aa/ n=54 analyzed hair cells for each genotype. (R) Knockdown of cdk5 rescues ER-stress-induced cell death in the ush1c mutant. Bars show average number of TUNEL-positive cells. For ush1c siblings n=52; ush1c siblings + 4 ng cdk5-MO n=18; ush1c/ n=70; ush1c/ + 4 ng cdk5-MO n=70 analyzed anterior maculae. Average ± s.d. Statistics were conducted with Student’s t-test. **P<0.01. NS, non significant. Some individual hair cells are outlined by dotted lines. MO, morpholino. Scale bar: 5 µm.

Gene Expression Details
Gene Antibody Fish Conditions Stage Qualifier Anatomy Assay
adgrv1 WT standard conditions Day 5 hair cell IHC
cdh23tj264/tj264 standard conditions Day 5 Not Detected hair cell IHC
ift88tz288/tz288 standard conditions Day 5 hair cell IHC
myo7aaty220/ty220 standard conditions Day 5 hair cell IHC
ush1cfh293/fh293 standard conditions Day 5 hair cell IHC
whrnb WT standard conditions Day 5 hair cell IHC
cdh23tj264/tj264 standard conditions Day 5 hair cell IHC
ift88tz288/tz288 standard conditions Day 5 hair cell IHC
myo7aaty220/ty220 standard conditions Day 5 hair cell IHC
ush1cfh293/fh293 standard conditions Day 5 hair cell IHC
Antibody Labeling Details
Antibody Assay Fish Conditions Stage Qualifier Anatomy
Ab1-ush2a IHC WT standard conditions Day 5 hair cell
IHC cdh23tj264/tj264 standard conditions Day 5 Not Detected hair cell
IHC ift88tz288/tz288 standard conditions Day 5 hair cell
IHC myo7aaty220/ty220 standard conditions Day 5 hair cell
IHC ush1cfh293/fh293 standard conditions Day 5 hair cell
Phenotype Details No data available
Acknowledgments:
ZFIN wishes to thank the journal Disease models & mechanisms for permission to reproduce figures from this article. Please note that this material may be protected by copyright. Full text @ Dis. Model. Mech.