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Product Name Gemin 5 Chinese Name 脊髓性肌萎缩症蛋白Gemin5抗体 Alias Gemin5; Gemin-5; gem (nuclear organelle) associated protein 5; Gem-associated protein 5; GEMIN5; GEMI5_HUMAN. Research Area Cell biology Neurobiology Epigenetics Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 169kDa Cellular localization The nucleus Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human Gemin 5: 401-500/1508 Lsotype IgG Purification affinity purified by Protein A Buffer Solution Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 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 Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord (1). SMA is caused by deletion or loss-of-function mutations in the SMN (Survival of Motor Neuron) gene (2). Gemin5, the protein product of human chromosome 5q33.3 (3), associates directly with SMN and is a part of the SMN complex containing Gemin2, Gemin3, Gemin4 and Gemin6 as well as several spliceosomal snRNP proteins (2,4). The SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus (2). The SMN complex is found in both the cytoplasm and the nucleus (2). The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies) (2,5,6). Gemin5 interacts with several snRNP core proteins including SmB, SmD1, SmD2, SmD3 and SmE (7). The amino terminal half of Gemin5 contains 13 WD repeat domains and a coiled-coil motif near the C-terminus (7).
Function:
Gemin 5 is part of a large macromolecular complex localized to both the cytoplasm and the nucleus that plays a role in the cytoplasmic assembly of small nuclear ribonucleoproteins (snRNPs). Other members of this complex include SMN, Gemin 2 (SIP1), Gemin 3 (DDX20), and Gemin 4.
Subunit:
Part of the core SMN complex that contains SMN1, SIP1/GEMIN2, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8 and STRAP/UNRIP. Interacts directly with SMN1, SNRPB, SNRPD1, SNRPD2, SNRPD3 and SNRPE.
Subcellular Location:
Nucleus, nucleoplasm. Nucleus, gem. Cytoplasm. Note=Found both in the nucleoplasm and in nuclear bodies called gems (Gemini of Cajal bodies) that are often in proximity to Cajal (coiled) bodies. Also found in the cytoplasm.
Similarity:
Belongs to the WD repeat gemin-5 family.
Contains 13 WD repeats.
SWISS:
Q8TEQ6
Gene ID:
25929
Database links:Entrez Gene: 25929 Human
Entrez Gene: 216766 Mouse
SwissProt: Q8TEQ6 Human
SwissProt: Q3UPH2 Mouse
SwissProt: Q8BX17 Mouse
Unigene: 483921 Human
Unigene: 275349 Mouse
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