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Katalogové číslo: (BOSSBS-9851R-A680)
Dodavatel: Bioss
Popis: The formation of the spliceosome includes the assembly of Sm proteins in an ordered manner onto snRNAs. This process is mediated by the survival of motor neuron (SMN) protein, and is enhanced by modification of specific arginine residues in the Sm proteins to symmetrical dimethylarginines (sDMAs). sDMA modification of Sm proteins is catalysed by the methylosome, a complex comprised of the type II methyltransferase PRMT5 (also designated Jak-binding protein 1, JBP1), pICln, and two novel factors. PRMT5 binds the Sm proteins via their arginine- and glycine-rich (RG) domains, while pICln binds the Sm domains. pICln also acts as an inhibitor of SnRNP assembly by preventing specific interactions between Sm proteins required for the formation of the Sm core. pICln is a highly conserved, ubiquitously expressed protein that localizes primarily to the cytoplasm, and may play a role as a swelling-activated anion channel or a channel regulator in addition to its function in the methylosome. The gene encoding human pICln maps to chromosome 11q14.1.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-9851R-A750)
Dodavatel: Bioss
Popis: The formation of the spliceosome includes the assembly of Sm proteins in an ordered manner onto snRNAs. This process is mediated by the survival of motor neuron (SMN) protein, and is enhanced by modification of specific arginine residues in the Sm proteins to symmetrical dimethylarginines (sDMAs). sDMA modification of Sm proteins is catalysed by the methylosome, a complex comprised of the type II methyltransferase PRMT5 (also designated Jak-binding protein 1, JBP1), pICln, and two novel factors. PRMT5 binds the Sm proteins via their arginine- and glycine-rich (RG) domains, while pICln binds the Sm domains. pICln also acts as an inhibitor of SnRNP assembly by preventing specific interactions between Sm proteins required for the formation of the Sm core. pICln is a highly conserved, ubiquitously expressed protein that localizes primarily to the cytoplasm, and may play a role as a swelling-activated anion channel or a channel regulator in addition to its function in the methylosome. The gene encoding human pICln maps to chromosome 11q14.1.
Měrná jednotka: 1 * 100 µl


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Katalogové číslo: (BOSSBS-11561R-A750)
Dodavatel: Bioss
Popis: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterised by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations of SMN (survival of motor neuron) gene. SMN, also known as Gemin1, SMN1, SMNT and BCD541, exists as four isoforms produced by alternative splicing. SMN is oligomeric and forms a complex with Gemin2 (formerly SIP1), Gemin3 (a DEAD box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. The SMN complex is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (gemini of the coiled bodies). Cytoplasmic SMN interacts with spliceosomal Sm proteins and facilitates their assembly onto U snRNAs, and nuclear SMN mediates recycling of pre-mRNA splicing factors. Nearly identical telomeric and centromeric forms of SMN encode the same protein; however, only mutations in the telomeric form are associated with the disease-state SMA. SMN is expressed in a wide variety of tissues including brain, kidney, liver, spinal cord and moderately in skeletal and cardiac muscle.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-11562R-HRP)
Dodavatel: Bioss
Popis: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations in the SMN (survival of motor neuron) gene. Gemin2 (formerly known as SIP1 for SMN interacting protein) associates directly with SMN and is a part of the SMN complex containing Gemin3 (a DEAD-box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. It is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies). The SMN-Gemin2 complex is associated with spliceosomal snRNAs U1 and U5. Gemin2 is expressed in spinal cord. It can be induced by TGF∫ treatment and expression is high in several E-cadherin negative human carcinoma cell lines. SMN is expressed in a wide variety of tissues including brain, kidney, liver and spinal cord, and moderately in skeletal and cardiac muscle. The gene encoding Gemin2 maps to human chromosome 14q13.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-11562R-A350)
Dodavatel: Bioss
Popis: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations in the SMN (survival of motor neuron) gene. Gemin2 (formerly known as SIP1 for SMN interacting protein) associates directly with SMN and is a part of the SMN complex containing Gemin3 (a DEAD-box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. It is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies). The SMN-Gemin2 complex is associated with spliceosomal snRNAs U1 and U5. Gemin2 is expressed in spinal cord. It can be induced by TGF∫ treatment and expression is high in several E-cadherin negative human carcinoma cell lines. SMN is expressed in a wide variety of tissues including brain, kidney, liver and spinal cord, and moderately in skeletal and cardiac muscle. The gene encoding Gemin2 maps to human chromosome 14q13.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-13330R-A555)
Dodavatel: Bioss
Popis: Gemin7 is a 131 amino acid protein encoded by the mouse gene Gemin7. Gemin7, along with Gemin6, is a significant component of the the large multiprotein human SMN complex. The SMN complex functions as an assembly machine for small nuclear ribonucleoproteins (snRNPs)-the major components of the spliceosome. The survival of motor neurons (SMN) protein, a product of the disease gene of the common neurodegenerative disease, spinal muscular atrophy, is also part of the SMN complex. Although Gemin6 and Gemin7 have no significant sequence similarity with Sm proteins, both adopt canonical Sm folds. Moreover, Gemin6 and Gemin7 exist as a heterodimer, and interact with each other via an interface similar to that which mediates interactions among the Sm proteins. The Gemin6/Gemin7 complex binds to Sm proteins and might help organize Sm proteins for formation of Sm rings on snRNA targets.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-13330R-A488)
Dodavatel: Bioss
Popis: Gemin7 is a 131 amino acid protein encoded by the mouse gene Gemin7. Gemin7, along with Gemin6, is a significant component of the the large multiprotein human SMN complex. The SMN complex functions as an assembly machine for small nuclear ribonucleoproteins (snRNPs)-the major components of the spliceosome. The survival of motor neurons (SMN) protein, a product of the disease gene of the common neurodegenerative disease, spinal muscular atrophy, is also part of the SMN complex. Although Gemin6 and Gemin7 have no significant sequence similarity with Sm proteins, both adopt canonical Sm folds. Moreover, Gemin6 and Gemin7 exist as a heterodimer, and interact with each other via an interface similar to that which mediates interactions among the Sm proteins. The Gemin6/Gemin7 complex binds to Sm proteins and might help organize Sm proteins for formation of Sm rings on snRNA targets.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-13330R-A350)
Dodavatel: Bioss
Popis: Gemin7 is a 131 amino acid protein encoded by the mouse gene Gemin7. Gemin7, along with Gemin6, is a significant component of the the large multiprotein human SMN complex. The SMN complex functions as an assembly machine for small nuclear ribonucleoproteins (snRNPs)-the major components of the spliceosome. The survival of motor neurons (SMN) protein, a product of the disease gene of the common neurodegenerative disease, spinal muscular atrophy, is also part of the SMN complex. Although Gemin6 and Gemin7 have no significant sequence similarity with Sm proteins, both adopt canonical Sm folds. Moreover, Gemin6 and Gemin7 exist as a heterodimer, and interact with each other via an interface similar to that which mediates interactions among the Sm proteins. The Gemin6/Gemin7 complex binds to Sm proteins and might help organize Sm proteins for formation of Sm rings on snRNA targets.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-13330R-A680)
Dodavatel: Bioss
Popis: Gemin7 is a 131 amino acid protein encoded by the mouse gene Gemin7. Gemin7, along with Gemin6, is a significant component of the the large multiprotein human SMN complex. The SMN complex functions as an assembly machine for small nuclear ribonucleoproteins (snRNPs)-the major components of the spliceosome. The survival of motor neurons (SMN) protein, a product of the disease gene of the common neurodegenerative disease, spinal muscular atrophy, is also part of the SMN complex. Although Gemin6 and Gemin7 have no significant sequence similarity with Sm proteins, both adopt canonical Sm folds. Moreover, Gemin6 and Gemin7 exist as a heterodimer, and interact with each other via an interface similar to that which mediates interactions among the Sm proteins. The Gemin6/Gemin7 complex binds to Sm proteins and might help organize Sm proteins for formation of Sm rings on snRNA targets.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-13330R-CY5)
Dodavatel: Bioss
Popis: Gemin7 is a 131 amino acid protein encoded by the mouse gene Gemin7. Gemin7, along with Gemin6, is a significant component of the the large multiprotein human SMN complex. The SMN complex functions as an assembly machine for small nuclear ribonucleoproteins (snRNPs)-the major components of the spliceosome. The survival of motor neurons (SMN) protein, a product of the disease gene of the common neurodegenerative disease, spinal muscular atrophy, is also part of the SMN complex. Although Gemin6 and Gemin7 have no significant sequence similarity with Sm proteins, both adopt canonical Sm folds. Moreover, Gemin6 and Gemin7 exist as a heterodimer, and interact with each other via an interface similar to that which mediates interactions among the Sm proteins. The Gemin6/Gemin7 complex binds to Sm proteins and might help organize Sm proteins for formation of Sm rings on snRNA targets.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-13330R-A647)
Dodavatel: Bioss
Popis: Gemin7 is a 131 amino acid protein encoded by the mouse gene Gemin7. Gemin7, along with Gemin6, is a significant component of the the large multiprotein human SMN complex. The SMN complex functions as an assembly machine for small nuclear ribonucleoproteins (snRNPs)-the major components of the spliceosome. The survival of motor neurons (SMN) protein, a product of the disease gene of the common neurodegenerative disease, spinal muscular atrophy, is also part of the SMN complex. Although Gemin6 and Gemin7 have no significant sequence similarity with Sm proteins, both adopt canonical Sm folds. Moreover, Gemin6 and Gemin7 exist as a heterodimer, and interact with each other via an interface similar to that which mediates interactions among the Sm proteins. The Gemin6/Gemin7 complex binds to Sm proteins and might help organize Sm proteins for formation of Sm rings on snRNA targets.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-11562R-FITC)
Dodavatel: Bioss
Popis: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations in the SMN (survival of motor neuron) gene. Gemin2 (formerly known as SIP1 for SMN interacting protein) associates directly with SMN and is a part of the SMN complex containing Gemin3 (a DEAD-box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. It is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies). The SMN-Gemin2 complex is associated with spliceosomal snRNAs U1 and U5. Gemin2 is expressed in spinal cord. It can be induced by TGF∫ treatment and expression is high in several E-cadherin negative human carcinoma cell lines. SMN is expressed in a wide variety of tissues including brain, kidney, liver and spinal cord, and moderately in skeletal and cardiac muscle. The gene encoding Gemin2 maps to human chromosome 14q13.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-11562R-CY5)
Dodavatel: Bioss
Popis: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations in the SMN (survival of motor neuron) gene. Gemin2 (formerly known as SIP1 for SMN interacting protein) associates directly with SMN and is a part of the SMN complex containing Gemin3 (a DEAD-box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. It is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies). The SMN-Gemin2 complex is associated with spliceosomal snRNAs U1 and U5. Gemin2 is expressed in spinal cord. It can be induced by TGF∫ treatment and expression is high in several E-cadherin negative human carcinoma cell lines. SMN is expressed in a wide variety of tissues including brain, kidney, liver and spinal cord, and moderately in skeletal and cardiac muscle. The gene encoding Gemin2 maps to human chromosome 14q13.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-11562R-CY7)
Dodavatel: Bioss
Popis: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations in the SMN (survival of motor neuron) gene. Gemin2 (formerly known as SIP1 for SMN interacting protein) associates directly with SMN and is a part of the SMN complex containing Gemin3 (a DEAD-box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. It is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies). The SMN-Gemin2 complex is associated with spliceosomal snRNAs U1 and U5. Gemin2 is expressed in spinal cord. It can be induced by TGF∫ treatment and expression is high in several E-cadherin negative human carcinoma cell lines. SMN is expressed in a wide variety of tissues including brain, kidney, liver and spinal cord, and moderately in skeletal and cardiac muscle. The gene encoding Gemin2 maps to human chromosome 14q13.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-11562R-CY3)
Dodavatel: Bioss
Popis: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations in the SMN (survival of motor neuron) gene. Gemin2 (formerly known as SIP1 for SMN interacting protein) associates directly with SMN and is a part of the SMN complex containing Gemin3 (a DEAD-box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. It is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies). The SMN-Gemin2 complex is associated with spliceosomal snRNAs U1 and U5. Gemin2 is expressed in spinal cord. It can be induced by TGF∫ treatment and expression is high in several E-cadherin negative human carcinoma cell lines. SMN is expressed in a wide variety of tissues including brain, kidney, liver and spinal cord, and moderately in skeletal and cardiac muscle. The gene encoding Gemin2 maps to human chromosome 14q13.
Měrná jednotka: 1 * 100 µl


Katalogové číslo: (BOSSBS-11561R-A680)
Dodavatel: Bioss
Popis: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterised by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations of SMN (survival of motor neuron) gene. SMN, also known as Gemin1, SMN1, SMNT and BCD541, exists as four isoforms produced by alternative splicing. SMN is oligomeric and forms a complex with Gemin2 (formerly SIP1), Gemin3 (a DEAD box RNA helicase), Gemin4, Gemin5 and Gemin6, as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in splicesomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. The SMN complex is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (gemini of the coiled bodies). Cytoplasmic SMN interacts with spliceosomal Sm proteins and facilitates their assembly onto U snRNAs, and nuclear SMN mediates recycling of pre-mRNA splicing factors. Nearly identical telomeric and centromeric forms of SMN encode the same protein; however, only mutations in the telomeric form are associated with the disease-state SMA. SMN is expressed in a wide variety of tissues including brain, kidney, liver, spinal cord and moderately in skeletal and cardiac muscle.
Měrná jednotka: 1 * 100 µl


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