<p>(A) Reduced HGS expression results in clawed paws in <i>Hgs</i><sup><i>tn/tn</i></sup> mice. Behavioral assays of (B) open field, (C) rotarod, (D) elevated beam, (E) von Frey and (F) forelimb grip strength for <i>Hgs</i><sup><i>+/+</i></sup> (black), <i>Hgs</i><sup><i>tn/+</i></sup>, <i>Hgs</i><sup><i>tn/tn</i></sup> and <i>Hgs</i><sup>KO/+</sup> mice. n = 6 mice per genotype for all assays except rotarod, where n = 4. Symbols represent unpaired t-tests corrected for multiple comparisons using the Holm-Sidak method. Data are shown as mean ± SE and n>6 animals per genotype for all assays except rotarod, where n = 4. A two-way anova demonstrated a significant effect of genotype on rotarod and elevated beam performance. Data are shown as me...
<p><i>HdhQ111</i>/+ and Hdh+/+ mice at 46 weeks of age were tested on an automated CatWalk system (A...
<p>(A) Representative immunoblot and (B) quantitation of the ESCRT-0 proteins HGS and STAM1, the ESC...
Includes bibliographical references (pages 48-53)Hereditary Inclusion Body Myopathy (HIBM) is a homo...
<p>(A) Wet weights of gastrocnemius muscles from 4 week-old <i>Hgs</i><sup><i>+/+</i></sup> and <i>H...
Includes bibliographical references (pages 46-52)Hereditary Inclusion Body Myopathy (HIBM) is a diso...
<p>A) Grip strength was assessed in GIT1 WT (n = 9, black bars) and GIT1 KO (n = 9, white bars) mice...
<p>(A) Image showing reduced size of 4-week-old <i>Hgs</i><sup><i>tn/tn</i></sup> mice relative to w...
<p>Both male (A) and female (B) <i>Hdh</i>Q150 mice performed better on the rotarod compared to cont...
<p>(A) Representative immunoblot of ubiquitin conjugates from the nervous system of 4-week-old <i>Hg...
<p>A) and B) Forelimbs strength assessment by grip test. A) WT and Hp<sup>-/-</sup> mice show overla...
<p>Effects on rotarod performance are assessed between days 35 to 60, analysis of hind-limb muscle v...
(A&B) Quantification of RotaRod studies. (A) Female Gnao1+/G184S mice lose the ability to stay on a ...
<p><b>A-C</b>. Mice were evaluated for motor functioning in a round beam apparatus. Compared to non-...
<p>(<b>A</b>) 1-d-old hSCARB2-Tg mice were injected s.c. with 1×10<sup>7</sup> pfu of E59 (B4) or th...
<p>A) Six month old female mice had no difference in grip strength between genotypes when normalized...
<p><i>HdhQ111</i>/+ and Hdh+/+ mice at 46 weeks of age were tested on an automated CatWalk system (A...
<p>(A) Representative immunoblot and (B) quantitation of the ESCRT-0 proteins HGS and STAM1, the ESC...
Includes bibliographical references (pages 48-53)Hereditary Inclusion Body Myopathy (HIBM) is a homo...
<p>(A) Wet weights of gastrocnemius muscles from 4 week-old <i>Hgs</i><sup><i>+/+</i></sup> and <i>H...
Includes bibliographical references (pages 46-52)Hereditary Inclusion Body Myopathy (HIBM) is a diso...
<p>A) Grip strength was assessed in GIT1 WT (n = 9, black bars) and GIT1 KO (n = 9, white bars) mice...
<p>(A) Image showing reduced size of 4-week-old <i>Hgs</i><sup><i>tn/tn</i></sup> mice relative to w...
<p>Both male (A) and female (B) <i>Hdh</i>Q150 mice performed better on the rotarod compared to cont...
<p>(A) Representative immunoblot of ubiquitin conjugates from the nervous system of 4-week-old <i>Hg...
<p>A) and B) Forelimbs strength assessment by grip test. A) WT and Hp<sup>-/-</sup> mice show overla...
<p>Effects on rotarod performance are assessed between days 35 to 60, analysis of hind-limb muscle v...
(A&B) Quantification of RotaRod studies. (A) Female Gnao1+/G184S mice lose the ability to stay on a ...
<p><b>A-C</b>. Mice were evaluated for motor functioning in a round beam apparatus. Compared to non-...
<p>(<b>A</b>) 1-d-old hSCARB2-Tg mice were injected s.c. with 1×10<sup>7</sup> pfu of E59 (B4) or th...
<p>A) Six month old female mice had no difference in grip strength between genotypes when normalized...
<p><i>HdhQ111</i>/+ and Hdh+/+ mice at 46 weeks of age were tested on an automated CatWalk system (A...
<p>(A) Representative immunoblot and (B) quantitation of the ESCRT-0 proteins HGS and STAM1, the ESC...
Includes bibliographical references (pages 48-53)Hereditary Inclusion Body Myopathy (HIBM) is a homo...