| Cajal body-like structures called "gems"
(gemini satellites of Cajal body) have recently been shown to be
paired frequently with Cajal bodies. Sometimes the two structures
overlap, and both are similarly affected by cell growth temperature
and transcription inhibitors.
Gems contain the SMN (survival of motor neurons) protein, encoded
by the gene responsible for a severe inherited form of human muscular
wasting disease, spinal muscular atrophy. The SMN protein interacts
with the Sm class of snRNP proteins and (through a separate binding
site) with a cellular protein called SIP1. The SMN-SIP1 complex
plays an essential role in cytoplasmic snRNP biogenesis.
Defects in spliceosomal snRNP assembly may thus be involved in spinal
muscular atrophy, and an intranuclear snRNP trafficking pathway
may involve interactions between gems and Cajal bodies.
 |
HeLa cells stained with
MANSMA I*, a monoclonal antibody to SMN.
*Young, P.J., Le, T.T., thi Man, N., Burghes, A.H. and Morris,
G.E. (2000) Exp. Cell Res. 256:365-374.
image provide by Dr. Judith Sleeman |
|