Product Name	NOL5A/NOP56
Chinese Name	核仁蛋白5A抗体
Alias	NOL5A; NOP56; NOP56 ribonucleoprotein; NOP56 ribonucleoprotein homolog (yeast); NOP56 ribonucleoprotein homolog; Nucleolar protein 56; Nucleolar protein 5A (56kD with KKE/D repeat); Nucleolar protein 5A; Nucleolar protein Nop56; NOP56_HUMAN; RP4-686C3.1; SCA36.
Research Area	Cell biology  Chromatin and nuclear signals  transcriptional regulatory factor  Epigenetics
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse,  (predicted: Human, Rat, Chicken, Pig, )
Applications	ELISA=1:5000-10000  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	66kDa
Cellular localization	The nucleus
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human NOL5A/NOP56: 301-400/594
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	NOL5A is similar in sequence to Nop56p, a yeast nucleolar protein that is part of a complex with the nucleolar proteins Nop58p and fibrillarin. Like Nop56p, NOL5A is found in the nucleolus and is though to be required for 60S ribosomal subunit biogenesis. Multiple transcript variants encoding several isoforms have been identified for this gene, but the full-length nature of most of them is not yet determined. Function: Involved in the early to middle stages of 60S ribosomal subunit biogenesis. Core component of box C/D small nucleolar ribonucleoprotein (snoRNP) particles. Required for the biogenesis of box C/D snoRNAs such U3, U8 and U14 snoRNAs. Subcellular Location: Nuclear; nucleolus DISEASE: Spinocerebellar ataxia 36 (SCA36) [MIM:614153]: A form of spinocerebellar ataxia, a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA36 is characterized by complicated clinical features, with ataxia as the first symptom, followed by characteristic late-onset involvement of the motor neuron system. Ataxic symptoms, such as gait and truncal instability, ataxic dysarthria, and uncoordinated limbs, start in late forties to fifties. Characteristically, affected individuals exhibit tongue atrophy with fasciculation. Progression of motor neuron involvement is typically limited to the tongue and main proximal skeletal muscles in both upper and lower extremities. Note: The disease is caused by mutations affecting the gene represented in this entry. Caused by large hexanucleotide CGCCTG repeat expansions within intron 1. These expansions induce RNA foci and sequester the RNA-binding protein SRSF2. In addition, the transcription of MIR1292, a microRNA gene located just 19 bp 3' of the GGCCTG repeat, is significantly decreased. Similarity: Belongs to the NOP5/NOP56 family. {ECO:0000305}. Contains 1 Nop domain. SWISS: O00567 Gene ID: 10528 Database links: Entrez Gene: 10528 Human Entrez Gene: 67134 Mouse Entrez Gene: 362214 Rat Omim: 614154 Human SwissProt: O00567 Human SwissProt: Q9D6Z1 Mouse Unigene: 145204 Rat

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