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Ace+Method+Development+Kits
Numéro de catalogue:
(BOSSBS-9631R-A350)
Fournisseur:
Bioss
Description:
Involvement in disease;Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-11510R-A350)
Fournisseur:
Bioss
Description:
Bardet-Biedl syndrome (BBS) is a pleiotropic genetic disorder characterized by obesity, photoreceptor degeneration, polydactyly, hypogenitalism, renal abnormalities, and developmental delay. BBS patients also have an increased risk of developing diabetes, hypertension, and congenital heart defects. BBS is a heterogeneous disorder mapping to eight genetic loci and encoding eight proteins, BBS1-BBS8. Five BBS proteins encode basal body or cilia proteins, suggesting that BBS is a ciliary dysfunction disorder. BBS2 contains two overlapping genes: BBS2L1 and BBS2L2. BBSL1 was re-named BBS7, whereas BBS2L2 independently funcitons as BBS1. BBS7 contains 672 amino acids and is expressed at low to moderate levels in most human tissues.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-5617R-HRP)
Fournisseur:
Bioss
Description:
Smad3 is a 50 kDa member of a family of proteins that act as key mediators of TGF beta superfamily signaling in cell proliferation, differentiation and development. The Smad family is divided into three subclasses: receptor regulated Smads, activin/TGF beta receptor regulated (Smad2 and 3) or BMP receptor regulated (Smad 1, 5, and 8); the common partner, (Smad4) that functions via its interaction to the various Smads; and the inhibitory Smads, (Smad6 and 7). Activated Smad3 oligomerizes with Smad4 upon TGF beta stimulation and translocates as a complex into the nucleus, allowing its binding to DNA and transcription factors. Phosphorylation of the two TGF beta dependent serines 423 and 425 in the C terminus of Smad3 is critical for Smad3 transcriptional activity and TGF beta signaling.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-4173R-A750)
Fournisseur:
Bioss
Description:
TWIST1 and TWIST2 are basic helix-loop-helix transcriptional repressors that bind to E boxes in gene promoters, acting as master regulators in a variety of biological processes, including organogenesis, osteogenesis, cancer progression and hematopoietic cell development. Both TWIST1 and TWIST2 are found to act as Homodimers or Heterodimers with another bHLH protein. TWIST1 Acts as a transcriptional regulator. Inhibits myogenesis by sequestrating E proteins, inhibiting trans-activation by MEF2, and inhibiting DNA-binding by MYOD1 through physical interaction. TWIST1 also represses expression of proinflammatory cytokines such as TNFA and IL1B. TWIST2 inhibits transcriptional activation by MYOD1, MYOG, MEF2A, and MEF2C. Twist2 inhibits the premature or ectopic differentiation of preosteoblast cells during osteogenesis.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-1961R)
Fournisseur:
Bioss
Description:
May act in negative regulation of cell growth and transformation by interacting with nonreceptor tyrosine kinases ABL1 and/or ABL2. May play a role in regulation of EGF-induced Erk pathway activation. Involved in cytoskeletal reorganization and EGFR signaling. Together with EPS8 participates in transduction of signals from Ras to Rac. In vitro, a trimeric complex of ABI1, EPS8 and SOS1 exhibits Rac specific guanine nucleotide exchange factor (GEF) activity and ABI1 seems to act as an adapter in the complex. Regulates ABL1/c-Abl-mediated phosphorylation of ENAH. Recruits WASF1 to lamellipodia and there seems to regulate WASF1 protein level. In brain, seems to regulate the dendritic outgrowth and branching as well as to determine the shape and number of synaptic contacts of developing neurons.
UOM:
1 * 100 µl
Fournisseur:
Biotium
Description:
Reacts with a protein of 57 kDa, identified as the L1 protein of human papilloma virus type 16 (HPV-16). It is the major capsid protein of HPV-16. Infection with specific types of HPV has been associated with an increased risk of developing cervical neoplasia. HPV types 6 and 11 have been associated with relatively benign diseases such as genital warts but types 16 and 18 are strongly associated with cervical, vaginal, and vulvar malignancies. The antibody reacts very strongly with formalin-fixed, paraffin-embedded tissues containing HPV-16 or -33; very weak reactions were occasionally observed with biopsy specimens or smears containing HPV-6 or HPV-11. It cross-reacts with HPV37.
Numéro de catalogue:
(BOSSBS-13179R)
Fournisseur:
Bioss
Description:
FKBP8 is an immunophilin family member lacking PPIase/arotamase activity that influences immunoregulation, protein folding and trafficking in neurons associated with memory function. The FKBPr38 form derives from a truncated ORF. Presenilin 1 and 2 form molecular complexes withæ¢nd promote degradation ofæ¡KBPr38, and Bcl-2, and sequester these proteins in ER/Golgi, thereby inhibiting FKBPr38-mediated, ?secretase-independent, mitochondrial targeting of Bcl-2. FKBP8 present in the central nervous system can antagonize hedgehog (HH) signaling, where HH is critical for patterning and growth of many tissues in the developing embryo. Mouse FKBPr38 mRNA is present in neurons and glial cells and appears more pronounced in neurons associated with the hippocampal formation in adult mouse brains.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-11830R-A647)
Fournisseur:
Bioss
Description:
Carbonic anhydrases (CAs) are members of a large family of zinc metalloenzymes responsible for catalyzing the reversible hydration of carbon dioxide. CAs show extensive diversity in their distribution and subcellular localization. They are involved in a variety of biological processes, including calcification, bone resorption, respiration, acid-base balance and the formation of aqueous humor, saliva, gastric juice and cerebrospinal fluid. CA X also referred to as Carbonic anhydrase-related protein X (CA-RP X) or Cerebral protein 15, is a member of the carbonic anhydrase family that lacks two of the three Zn-binding motifs essential for carbonic anhydrase activity. For this reason, CA X does not exhibit catalytic activity. It is expressed primarily in brain and kidney and may play a role in brain development.
UOM:
1 * 100 µl
Fournisseur:
SGE a Trajan company
Description:
SGE a évalué chaque méthode ASTM et EN pour biodiesel afin de démontrer la capacité des colonnes BPX‑BIOD GC à offrir une séparation optimale.
Fournisseur:
TCI
Description:
N-Acetyl-D-phenylalanine ≥98.0% (par HPLC, analyse par titration)
Numéro de catalogue:
(BOSSBS-2439R-CY3)
Fournisseur:
Bioss
Description:
Component of the beta-catenin destruction complex required for regulating CTNNB1 levels through phosphorylation and ubiquitination, and modulating Wnt-signaling. Controls dorsoventral patterning via two opposing effects; down-regulates CTNNB1 to inhibit the Wnt signaling pathway and ventralize embryos, but also dorsalizes embryos by activating a Wnt-independent JNK signaling pathway. In Wnt signaling, probably facilitates the phosphorylation of CTNNB1 and APC by GSK3B. Likely to function as a tumor suppressor. Facilitates the phosphorylation of TP53 by HIPK2 upon ultraviolet irradiation. Enhances TGF-beta signaling by recruiting the RNF111 E3 ubiquitin ligase and promoting the degradation of inhibitory SMAD7. Also component of the AXIN1-HIPK2-TP53 complex which controls cell growth, apoptosis and development.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-9421R-CY3)
Fournisseur:
Bioss
Description:
NET-6, also known as TSPAN13 (tetraspanin-13) or TM4SF13 (transmembrane 4 superfamily member 13), is a 204 amino acid multi-pass membrane protein that belongs to the tetraspanin (TM4SF) family. Members of the tetraspanin family are cell-surface proteins that are characterized by the presence of four hydrophobic domains and mediate signal transduction events that play a role in the regulation of cell development, activation, growth, motility, differentiation, and cancer. Considered molecular facilitators, tetraspanin proteins may regulate vesicle fusion and fission. The gene encoding NET-6 maps to human chromosome 7, which houses over 1,000 genes, comprises nearly 5% of the human genome and has been linked to Osteogenesis imperfecta, Pendred syndrome, Lissencephaly, Citrullinemia and Shwachman-Diamond syndrome.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-9479R-CY5)
Fournisseur:
Bioss
Description:
SMARCD3, is a member of the SMARCD family and contains one SWIB domain. Two isoforms, isoform 1 and isoform 2 exist due to alternative splicing events. Both isoforms are expressed in placenta, salivary gland, kidney, brain, trachea, uterus, prostate, testis, thyroid, spleen and heart, while isoform 1 is also expressed in adipose tissue and skeletal muscle. Localizing to the nucleus, SMARCD3 is a component of the ATP-dependent chromatin remodeling complex SNF/SWI and is believed to play a role in nucleosome remodeling. SMARCD3 also plays an important role in the regulation of muscle development. In mice, the silencing of the gene en-coding SMARCD3 leads to defects in heart morphogenesis. In addition, both isoforms of SMARCD3 directly interact with and function as coactivators for several transcription factors.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-9448R-CY3)
Fournisseur:
Bioss
Description:
The tetraspanin family is a group of cell surface proteins that regulate cell development, activation, growth and motility. Each member contains four hydrophobic domains and participates in the mediation of signal transduction. NET-5, also known as TSPAN9 (tetraspanin 9), is a 239 amino acid multi-pass membrane protein that belongs to the tetraspanin (TM4SF) family. NET-5 forms a complex with GPVI in the tetraspanin microdomains on the platelet surface, and is encoded by a gene that maps to human chromosome 12p13.33. Chromosome 12 encodes over 1,100 genes and comprises approximately 4.5% of the human genome. Chromosome 12 is associated with a variety of diseases and afflictions, including hypochondrogenesis, achondrogenesis, Kniest dysplasia, Noonan syndrome and trisomy 12p, which causes facial developmental defects and seizure disorders.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-11340R-CY5)
Fournisseur:
Bioss
Description:
The regulated translation of messenger RNA is essential for cell-cycle progression, establishment of the body plan during early development and modulation of key activities in the central nervous system. Cytoplasmic polyadenylation, one mechanism of controlling translation, is driven by cytoplasmic polyadenylation element binding proteins, called CPEBs. CPEB3 (cytoplasmic polyadenylation element binding protein 3) is a 698 amino acid protein that contains two RNA recognition motif (RRM) domains and, like other CPEB proteins, may play a role in the maturation of the central nervous system. CPEB3 exists as multiple alternatively spliced isoforms that are encoded by a gene which maps to human chromosome 10, which houses over 1,200 genes and comprises nearly 4.5% of the human genome.
UOM:
1 * 100 µl
Numéro de catalogue:
(BOSSBS-12873R-CY3)
Fournisseur:
Bioss
Description:
Bone morphogenic proteins (BMPs) are members of the TGF Beta superfamily. BMPs are involved in the induction of cartilage and bone formation. In vivo studies have shown that BMP-2 (also designated BMP-2A) and BMP-3 can independently induce cartilage formation. Smad3 association with the TGF Beta receptor complex and Smad1 translocation to the nucleus are observed after the addition of BMP-4 (also designated BMP-2B), suggesting that BMP-4 may play a role in activation of the Smad pathway. BMP-5, BMP-6 and BMP-7 all share high sequence homology with BMP-2, indicating that they each may be able to induce cartilage formation. BMP-8 (also designated OP-2) is thought to be involved in early development, as detectable expression has not been found in adult organs.
UOM:
1 * 100 µl
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