GLYCOCONJUGATES ASSOCIATED WITH SPINOCEREBELLAR NEURONS ARE IDENTIFIED BY MAB 6A2

1990 ◽  
Vol 49 (3) ◽  
pp. 278
Author(s):  
C. Williams ◽  
L. Reiter ◽  
G. Pegolo ◽  
S. Benzer ◽  
C. Miller
2005 ◽  
Vol 25 (24) ◽  
pp. 10953-10964 ◽  
Author(s):  
Chie Kaneko-Oshikawa ◽  
Tadashi Nakagawa ◽  
Mitsunori Yamada ◽  
Hiroo Yoshikawa ◽  
Masaki Matsumoto ◽  
...  

ABSTRACT Ubiquitin conjugation typically requires three classes of enzyme: E1, E2, and E3. A fourth type of enzyme (E4), however, was recently shown to be required for the degradation of certain types of substrate in yeast. We previously identified UFD2a (also known as E4B) as an E4 in mammals. UFD2a is exclusively expressed in cardiac muscle during mouse embryonic development, but it is abundant in neurons of adult mice and is implicated in the pathogenesis of neurodegenerative disease. The precise physiological function of this enzyme has remained largely unknown, however. Here, we show that mice lacking UFD2a die in utero, manifesting marked apoptosis in the developing heart. Polyubiquitylation activity for an E4 substrate was greatly reduced in Ufd2a −/− mouse embryonic fibroblasts. Furthermore, Ufd2a +/− mice displayed axonal dystrophy in the nucleus gracilis, as well as degeneration of Purkinje cells accompanied by endoplasmic reticulum stress. These animals also developed a neurological disorder. UFD2a thus appears to be essential for the development of cardiac muscle, as well as for the protection of spinocerebellar neurons from degeneration induced by endoplasmic reticulum stress.


2017 ◽  
Vol 145 ◽  
pp. S122
Author(s):  
Eamon Coughlan ◽  
Victoria C. Garside ◽  
Olivier Serralbo ◽  
Siew-Fen Lisa Wong ◽  
Huazheng Liang ◽  
...  

1977 ◽  
Vol 130 (1) ◽  
pp. 146-151 ◽  
Author(s):  
J.M. Petras

1990 ◽  
Vol 27 (3) ◽  
pp. 215-225 ◽  
Author(s):  
Celia Williams ◽  
Mark A. Kozlowski ◽  
David R. Hinton ◽  
Carol A. Miller

1993 ◽  
Vol 70 (2) ◽  
pp. 863-866 ◽  
Author(s):  
G. Bosco ◽  
R. E. Poppele

1. Spinocerebellar neurons that project in the dorsal spinocerebellar tract (DSCT) receive mono- and polysynaptic inputs from specific sensory receptors in the hindlimb, and they project mossy fiber terminals to the cerebellar vermis. We examined the functional organization of these neurons and found that it relates to whole-limb parameters like limb posture and direction of limb movement. 2. We recorded the activity of 444 DSCT units during passive perturbations of the hind foot in anesthetized cats. The movements were either confined a single joint (the ankle; 234 cells) or involved the entire hindlimb (210 cells). The cells exhibited opposite responses for opposite directions of whole-limb movement, but a variety of response patterns for opposite directions of movement at one joint. We interpret the result to imply that the population encodes information about the whole limb rather than single joints. 3. Most of the 78 neurons recorded during passive limb placements (63%) responded to changes in limb length and also changes in limb orientation. In fact, the activity of most of the cells was broadly tuned with respect to the direction of passive limb movements generated by moving the hind foot in the sagittal plane. Changes in unit activity could be described by a cosine tuning function with respect to foot positions (72% of responses) and directions of foot movement (50%). 4. The similarity of this behavior to that of neurons in the motor cortex and cerebellar nuclei recorded during voluntary movements is consistent with a common neural code to represent the sensorimotor parameters of limb movement.


2012 ◽  
Vol 74 (4) ◽  
pp. 495-498 ◽  
Author(s):  
Masato UEHARA ◽  
Masahide AKITA ◽  
Masato FURUE ◽  
Aya SHINOZAKI ◽  
Yoshinao Z. HOSAKA

Sign in / Sign up

Export Citation Format

Share Document