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Home / Primary Antibodies / Polyclonal Antibodies

SMN antibody

SKU : BHA10808022

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Mfr.No.	FNab08030
Alternative Names	CBCD541 ; Componentofgems1 ; FLJ76644 ; Gemin1 ; SMN ; SMN1 ; SMN1 ; SMN ; SMN2 ; SMNC ; SMNT
Background	This gene is part of a 500 kb inverted duplication on chromosome 5q13. This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. While mutations in the telomeric copy are associated with spinal muscular atrophy, mutations in this gene, the centromeric copy, do not lead to disease. This gene may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7, which is thought to be an exon splice enhancer. Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, 2a, 2b, and 3-8. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The full length protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Four transcript variants encoding distinct isoforms have been described.
Categories	Primary Antibodies
Clonality	polyclonal
Description	Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of anterior horn cells in the spinal cord and concomitant symmetrical muscle weakness and atrophy (PMID: 16364894 ). SMA is caused by deletion or mutations of the survival motor neuron (SMN1) gene. SMA patients lack a functional SMN1 gene, but they possess an intact SMN2 gene, which though nearly identical to SMN1, is only partially functional (PMID: 17355180). A large majority of SMN2 transcripts lack exon 7, resulting in production of a truncated, less stable SMN protein (PMID: 10369862). The level of SMN protein correlates with phenotypic severity of SMA. This antibody, 11708-1-AP, raised against the recombinant full-length human SMN2 protein, recognizes all isoforms of SMN protein.
Host	Rabbit
Immunogen	survival of motor neuron 2, centromeric
Isotype	IgG
Molecular Weight	38 kDa
Reactivity	Human, Mouse, Rat
Regulatory	RUO
Synonyms	C BCD541, Component of gems 1, FLJ76644, Gemin 1, SMN, SMN1, SMN1, SMN, SMN2, SMNC, SMNT, Survival motor neuron protein

Uniprot	Q16637
Gene Id	6607
Research Area	Metabolism, Neuroscience
Weight	38 kDa

Form	liquid
Format	liquid
Purification	Immunogen affinity purified
Purity	>=95% as determined by SDS-PAGE
Storage	PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20°C for 12 months (Avoid repeated freeze / thaw cycles.)

Applications

ELISA, IHC, IF, IP, WB

Description

ELISA ; IHC ; IF ; IP ; WB

Dilution

WB	1:500-1:5000

Tested Application

WB: 1:500 - 1:2000; IHC: 1:50 - 1:200

Manual	FNab08030.pdf

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SKU	Supplier No.	Size	Your price	Quantity
BHA10808022	FNab08030	100ug	$260		Add to Cart

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