U4atac minor spliceosomal RNA
Encyclopedia
U4atac minor spliceosomal RNA is a ncRNA which is an essential component of the minor U12-type spliceosome
Minor spliceosome
The minor spliceosome is a ribonucleoprotein complex that catalyses the removal of an atypical class of spliceosomal introns from eukaryotic messenger RNAs in plant, insects, vertebrates and some fungi . This process is called noncanonical splicing, as opposed to U2-dependent canonical splicing...

 complex. The U12-type spliceosome is required for removal of the rarer class of eukaryotic introns (AT-AC, U12-type).

U4atac snRNA is proposed to form a base-paired complex with another spliceosomal RNA U6atac
U6atac minor spliceosomal RNA
U6atac minor spliceosomal RNA is a non-coding RNA which is an essential component of the minor U12-type spliceosome complex. The U12-type spliceosome is required for removal of the rarer class of eukaryotic introns ....

 via two stem loop regions. These interacting stem loops have been shown to be required for in vivo
In vivo
In vivo is experimentation using a whole, living organism as opposed to a partial or dead organism, or an in vitro controlled environment. Animal testing and clinical trials are two forms of in vivo research...

 splicing
RNA splicing
In molecular biology and genetics, splicing is a modification of an RNA after transcription, in which introns are removed and exons are joined. This is needed for the typical eukaryotic messenger RNA before it can be used to produce a correct protein through translation...

. U4atac also contains an 3' Sm protein binding site which has been shown to be essential for splicing activity. U4atac is the functional analog of U4 spliceosomal RNA
U4 spliceosomal RNA
The U4 small nuclear Ribo-Nucleic Acid is a non-coding RNA component of the major or U2-dependent spliceosome – a eukaryotic molecular machine involved in the splicing of pre-messenger RNA...

 in the major U2-type spliceosomal
Spliceosome
A spliceosome is a complex of snRNA and protein subunits that removes introns from a transcribed pre-mRNA segment. This process is generally referred to as splicing.-Composition:...

 complex.

The Drosophila
Drosophila
Drosophila is a genus of small flies, belonging to the family Drosophilidae, whose members are often called "fruit flies" or more appropriately pomace flies, vinegar flies, or wine flies, a reference to the characteristic of many species to linger around overripe or rotting fruit...

 U4atac snRNA has an additional predicted 3' stem loop terminal to the Sm binding site.

Disease

It has been shown that mutations in the U4atac snRNA can cause microcephalic osteodysplastic primordial dwarfism type I
Primordial Dwarfism
Primordial dwarfism is a form of dwarfism that results in a smaller body size in all stages of life beginning from before birth. More specifically, primordial dwarfism is a diagnostic category including specific types of profoundly proportionate dwarfism, in which individuals are extremely small...

(MOPD I), also called Taybi-Linder syndrome (TALS). MOPD I is a developmental disorder that is associated with brain and skeletal abnormalities. It has been shown that the mutations cause defective U12 splicing.
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