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Artikel-Nr:
(BOSSBS-8071R)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-8071R
Lokale Artikelnummer::
BOSSBS-8071R
Beschreibung:
May be a chaperone-like protein essential for the proper conformation and functioning of protein complexes in the respiratory chain.Tissue specificity:Ubiquitously expressed with a relatively greater abundance in heart and skeletal muscle.Involvement in disease:Defects in ADCK3 are a cause of coenzyme Q10 deficiency (CoQ10 deficiency). CoQ10 deficiency is an autosomal recessive disorder with variable manifestations. It can be associated with three main clinical phenotypes: a predominantly myopathic form with central nervous system involvement, an infantile encephalomyopathy with renal dysfunction and an ataxic form with cerebellar atrophy.Defects in ADCK3 are the cause of spinocerebellar ataxia autosomal recessive type 9 (SCAR9) [MIM:612016]; also known as autosomal recessive cerebellar ataxia type 2 (ARCA2). Spinocerebellar ataxia is 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. SCAR9 is an autosomal recessive form characterized by gait ataxia and cerebellar atrophy with slow progression and few associated features. Patients can manifest brisk tendon reflexes and Hoffmann sign, mild psychomotor retardation, mild axonal degeneration of the sural nerve, exercise intolerance and elevated serum lactate.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-13340R-A680)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-13340R-A680
Lokale Artikelnummer::
BOSSBS-13340R-A680
Beschreibung:
Members of the mTERF family, including MTERF, MTERFD1, MTERFD2 and MTERFD3, are mitochondrial proteins that are believed to be transcription termination factors. MTERF (mitochondrial transcription termination factor 1) is composed of 399 amino acids and contains three leucine zippers that form a three-stranded coiled-coil that binds to DNA. It has been suggested that only the phosphorylated form of MTERF has transcription termination activity. MTERFD1 is also thought to act as a mitochondrial transcription regulator and is expressed as two isoforms produced by alternative splicing. MTERFD3 is believed to be involved in cell cycle regulation and cell growth by modulating mitochondrial transcription. MTERFD3 is expressed in heart, skeletal muscle, pancreas and liver.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-13340R-A555)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-13340R-A555
Lokale Artikelnummer::
BOSSBS-13340R-A555
Beschreibung:
Members of the mTERF family, including MTERF, MTERFD1, MTERFD2 and MTERFD3, are mitochondrial proteins that are believed to be transcription termination factors. MTERF (mitochondrial transcription termination factor 1) is composed of 399 amino acids and contains three leucine zippers that form a three-stranded coiled-coil that binds to DNA. It has been suggested that only the phosphorylated form of MTERF has transcription termination activity. MTERFD1 is also thought to act as a mitochondrial transcription regulator and is expressed as two isoforms produced by alternative splicing. MTERFD3 is believed to be involved in cell cycle regulation and cell growth by modulating mitochondrial transcription. MTERFD3 is expressed in heart, skeletal muscle, pancreas and liver.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-11364R-A488)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-11364R-A488
Lokale Artikelnummer::
BOSSBS-11364R-A488
Beschreibung:
The protein encoded by this gene is a coiled-coil-forming protein that associates with the SNARE (soluble N-ethylmaleimide-sensitive fusion protein attachment protein receptor) complex of proteins and the BLOC-1 (biogenesis of lysosome-related organelles) complex. Biochemical studies have identified additional binding partners. As part of the SNARE complex, it is required for vesicle docking and fusion and regulates neurotransmitter release. The BLOC-1 complex is required for the biogenesis of specialized organelles such as melanosomes and platelet dense granules. Mutations in gene products that form the BLOC-1 complex have been identified in mouse strains that are models of Hermansky-Pudlak syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2012].
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-9585R-A555)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-9585R-A555
Lokale Artikelnummer::
BOSSBS-9585R-A555
Beschreibung:
Crystallins are water soluble structural proteins found in the vertebrate eye. Mammalian crystallins are classified in three forms, designated α, β and γ. Crystallins, as the principal components of the lens, function to increase the refractive index of the eye during accommodation by forming high-molecular weight aggregates which maintain transparency. γS-crystallin (Gamma-crystallin S), also known as Beta-crystallin S, is a 178 amino acid protein that exists as a monomer which does not aggregate. γS-crystallin contains a two-domain beta structure and belongs to the beta/gamma-crystallin gene family mapping to human chromosome 3. γS-crystallin has been linked to congenital cataract development, a disorder signified by increasing levels of lens opacity.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-9585R-A350)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-9585R-A350
Lokale Artikelnummer::
BOSSBS-9585R-A350
Beschreibung:
Crystallins are water soluble structural proteins found in the vertebrate eye. Mammalian crystallins are classified in three forms, designated α, β and γ. Crystallins, as the principal components of the lens, function to increase the refractive index of the eye during accommodation by forming high-molecular weight aggregates which maintain transparency. γS-crystallin (Gamma-crystallin S), also known as Beta-crystallin S, is a 178 amino acid protein that exists as a monomer which does not aggregate. γS-crystallin contains a two-domain beta structure and belongs to the beta/gamma-crystallin gene family mapping to human chromosome 3. γS-crystallin has been linked to congenital cataract development, a disorder signified by increasing levels of lens opacity.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-11364R-A555)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-11364R-A555
Lokale Artikelnummer::
BOSSBS-11364R-A555
Beschreibung:
The protein encoded by this gene is a coiled-coil-forming protein that associates with the SNARE (soluble N-ethylmaleimide-sensitive fusion protein attachment protein receptor) complex of proteins and the BLOC-1 (biogenesis of lysosome-related organelles) complex. Biochemical studies have identified additional binding partners. As part of the SNARE complex, it is required for vesicle docking and fusion and regulates neurotransmitter release. The BLOC-1 complex is required for the biogenesis of specialized organelles such as melanosomes and platelet dense granules. Mutations in gene products that form the BLOC-1 complex have been identified in mouse strains that are models of Hermansky-Pudlak syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jun 2012].
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-9585R-CY7)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-9585R-CY7
Lokale Artikelnummer::
BOSSBS-9585R-CY7
Beschreibung:
Crystallins are water soluble structural proteins found in the vertebrate eye. Mammalian crystallins are classified in three forms, designated α, β and γ. Crystallins, as the principal components of the lens, function to increase the refractive index of the eye during accommodation by forming high-molecular weight aggregates which maintain transparency. γS-crystallin (Gamma-crystallin S), also known as Beta-crystallin S, is a 178 amino acid protein that exists as a monomer which does not aggregate. γS-crystallin contains a two-domain beta structure and belongs to the beta/gamma-crystallin gene family mapping to human chromosome 3. γS-crystallin has been linked to congenital cataract development, a disorder signified by increasing levels of lens opacity.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-11621R-A555)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-11621R-A555
Lokale Artikelnummer::
BOSSBS-11621R-A555
Beschreibung:
The family of guanylin regulatory peptides, including guanylin and uroguanylin, are strongly expressed in intestinal mucosa and regulate intestinal fluid secretion during digestion. Guanylins are also involved in acid neutralization and the regulation of membrane-bound guanylate cyclase signaling molecules. Guanylin and uroguanylin are secreted primarily in the stomach, intestine, and colon. Guanylin is also detected in plasma. Guanylin is an endogenous activator of intestinal guanylate cyclase. It stimulates intestinal guanylate cyclase through the same receptor binding region as the heat-stable enterotoxins. Gut enterochromaffin cells synthesize guanylin to be a prohormone of 115 amino acids which is then is processed to the molecular form of 94 amino acids. This 10kDa form is found circulating in the blood.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-5981R-A555)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-5981R-A555
Lokale Artikelnummer::
BOSSBS-5981R-A555
Beschreibung:
p107 is a tumor suppressor protein that appears to be involved in cell cycle regulation, as it is phosphorylated in the S to M phase transition and is dephosphorylated in the G1 phase of the cell cycle. Both the RB1 protein and the product of this gene can form a complex with adenovirus E1A protein and SV40 large T-antigen, with the SV40 large T-antigen binding only to the unphosphorylated form of each protein. In addition, both proteins can inhibit the transcription of cell cycle genes containing E2F binding sites in their promoters. Due to the sequence and biochemical similarities with the RB1 protein, it is thought that the protein encoded by this gene may also be a tumor suppressor. Two transcript variants encoding different isoforms have been found for this gene.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-15186R-A488)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-15186R-A488
Lokale Artikelnummer::
BOSSBS-15186R-A488
Beschreibung:
Non-enzymatic component of C3 and C5 convertases and thus essential for the propagation of the classical complement pathway. Covalently binds to immunoglobulins and immune complexes and enhances the solubilization of immune aggregates and the clearance of IC through CR1 on erythrocytes. C4A isotype is responsible for effective binding to form amide bonds with immune aggregates or protein antigens, while C4B isotype catalyzes the transacylation of the thioester carbonyl group to form ester bonds with carbohydrate antigens. Derived from proteolytic degradation of complement C4, C4a anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-5981R-A488)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-5981R-A488
Lokale Artikelnummer::
BOSSBS-5981R-A488
Beschreibung:
p107 is a tumor suppressor protein that appears to be involved in cell cycle regulation, as it is phosphorylated in the S to M phase transition and is dephosphorylated in the G1 phase of the cell cycle. Both the RB1 protein and the product of this gene can form a complex with adenovirus E1A protein and SV40 large T-antigen, with the SV40 large T-antigen binding only to the unphosphorylated form of each protein. In addition, both proteins can inhibit the transcription of cell cycle genes containing E2F binding sites in their promoters. Due to the sequence and biochemical similarities with the RB1 protein, it is thought that the protein encoded by this gene may also be a tumor suppressor. Two transcript variants encoding different isoforms have been found for this gene.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-12033R-CY3)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-12033R-CY3
Lokale Artikelnummer::
BOSSBS-12033R-CY3
Beschreibung:
Glutamate receptors mediate most excitatory neurotransmission in the brain and play an important role in neural plasticity, neural development and neurodegeneration. Ionotropic glutamate receptors are categorized into NMDA receptors and kainate/AMPA receptors, both of which contain glutamate-gated, cation-specific ion channels. Kainate/AMPA receptors are co-localized with NMDA receptors in many synapses and consist of the structurally related subunits GluR-1 to -7, KA1 and KA2. KA1 (also designated EEA1) and KA2 (also designated EEA2) form heteromeric receptors with GluR subunits when coexpressed, forming ion channels with various properties. The kainate/AMPA receptors are primarily responsible for the fast excitatory neuro-transmission by glutamate.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-5803R-CY7)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-5803R-CY7
Lokale Artikelnummer::
BOSSBS-5803R-CY7
Beschreibung:
Apoptotic suppressor. The BIR motifs region interacts with TNF receptor associated factors 1 and 2 (TRAF1 and TRAF2) to form an heteromeric complex, which is then recruited to the tumor necrosis factor receptor 2 (TNFR2).
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-1506R-CY5)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-1506R-CY5
Lokale Artikelnummer::
BOSSBS-1506R-CY5
Beschreibung:
Forms a water-specific channel that provides the plasma membranes of red cells and kidney proximal tubules with high permeability to water, thereby permitting water to move in the direction of an osmotic gradient.
VE:
1 * 100 µl
Artikel-Nr:
(BOSSBS-11017R-CY7)
Lieferant:
Bioss
Hersteller-Artikelnummer::
BS-11017R-CY7
Lokale Artikelnummer::
BOSSBS-11017R-CY7
Beschreibung:
Long pentraxins are a family of highly conserved proteins that are expressed in the brain and central nervous system, and form multimeric complexes. Neuronal pentraxin 1 (NP1), NP2, and neuronal pentraxin receptor (NPR) are members of the long pentraxins that represent a neuronal uptake pathway that may function during synapse formation and remodeling. The NP1 gene is located on chromosome 17q25.3 and the protein product mediates the uptake of synaptic material, including the presynaptic snake venom toxin, taipoxin (3). NP2, whose function is unknown, is located on chromosome 7q22.1 and like NP1 contains several potential N-linked glycosylation sites. NPR is expressed on the cell membrane and can form heteropentamers with NP1 and NP2 that can be released from the cell membrane by proteolysis.
VE:
1 * 100 µl
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 noch angezeigt wird und Sie Hilfe benötigen, rufen Sie uns an 1-800-932 - 5000.
Lager für diesen Artikel ist begrenzt, kann aber in einem Lagerhaus in Ihrer Nähe zur Verfügung. Bitte stellen Sie sicher, dass Sie in sind angemeldet auf dieser Seite, so dass verfügbare Bestand angezeigt werden können. Wenn das
noch angezeigt wird und Sie Hilfe benötigen, rufen Sie uns an 1-800-932 - 5000.
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