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3-Picolyl+isothiocyanate+hydrobromide
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3-Picolyl+isothiocyanate+hydrobromide
Catalog Number:
(10322-670)
Supplier:
Bioss
Description:
Component of the BLOC-1 complex, a complex that is required for normal biogenesis of lysosome-related organelles (LRO), such as platelet dense granules and melanosomes. In concert with the AP-3 complex, the BLOC-1 complex is required to target membrane protein cargos into vesicles assembled at cell bodies for delivery into neurites and nerve terminals. The BLOC-1 complex, in association with SNARE proteins, is also proposed to be involved in neurite extension. Associates with the BLOC-2 complex to facilitate the transport of TYRP1 independent of AP-3 function. Plays a role in synaptic vesicle trafficking and in neurotransmitter release. Plays a role in the regulation of cell surface exposure of DRD2. May play a role in actin cytoskeleton reorganization and neurite outgrowth. May modulate MAPK8 phosphorylation. Appears to promote neuronal transmission and viability through regulating the expression of SNAP25 and SYN1, modulating PI3-kinase-Akt signaling and influencing glutamatergic release. Regulates the expression of SYN1 through binding to its promoter. Modulates prefrontal cortical activity via the dopamine/D2 pathway.
Catalog Number:
(10285-552)
Supplier:
Bioss
Description:
Acid ceramidase catalyzes the degradation of ceramide in normal tissues, and deficiency leads to accumulation of ceramide in tissues, a hallmark of Farber disease. Effected individuals experience early onset joint problems and neurological problems, owing to mutations in the acid ceramidase gene. Bioinformatic analysis of gene expression also reveals acid ceramidase to be among the 5 most important genes associated with melanoma. In addition to ceramide hydrolysis, purified acid ceramidase also exhibits the ability to catalyze ceramide synthesis, utilizing [14C]lauric acid and sphingosine as substrates. Interestingly, pH regulates which reaction is favored; for hydrolysis the pH optimum is 4.5, whereas for the reverse reaction favors a pH of 5.5, further supporting a complex and central role for acid ceramidase in sphingolipid metabolism.
Catalog Number:
(10270-792)
Supplier:
Bioss
Description:
The leucine-rich (LRR) repeat is a 20-30 amino acid motif that forms a hydrophobic å/∫ horseshoe fold, allowing it to accommodate several leucine residues within a tightly packed core. All LRR repeats contain a variable segment and a highly conserved segment, the latter of which accounts for 11 or 12 residues of the entire LRR motif. SLITRK6 (SLIT and NTRK-like family, member 6) is an 841 amino acid single-pass type I membrane protein that contains 16 LRR repeats and belongs to the SLITRK family. Expressed at high levels in select brain tissue, as well as in both adult and fetal lung, SLITRK6 functions to suppress neurite outgrowth, playing a role in the regulation of neuronal function. Multiple isoforms of SLITRK6 exist due to alternative splicing events.
Catalog Number:
(10296-856)
Supplier:
Bioss
Description:
Growth hormone 2 (GH2) is a member of the somatotropin/prolactin family of hormones which play an important role in growth control. The gene, along with four other related genes, is located at the growth hormone locus on chromosome 17 where they are interspersed in the same transcriptional orientation; an arrangement which is thought to have evolved by a series of gene duplications. The five genes share a remarkably high degree of sequence identity. Mutations in this gene lead to placental growth hormone/lactogen deficiency. The major role of GH2 in stimulating body growth is to stimulate the liver and other tissues to secrete IGF-1. It stimulates both the differentiation and proliferation of myoblasts. It also stimulates amino acid uptake and protein synthesis in muscle and other tissues.
Catalog Number:
(10288-584)
Supplier:
Bioss
Description:
All members of the Src gene family of tyrosine kinases are characterized by a carboxy terminal domain tyrosine which is highly phosphorylated in the inactive form of the enzyme and phosphorylated to a much lesser extent when the enzyme is active. In the case of Src p60, Y527 is this tyrosine; however, a mutant form of c-Src in which Y527 is replaced by phenylalanine is transforming and displays 5- to 10-fold elevated kinase activity compared to its normal counterpart. Csk has been identified as a Src-related tyrosine kinase having both SH2 and SH3 domains and a catalytic domain but lacking sequences amino terminal to the SH3 domain as well as carboxy terminal regulatory sequences. Csk phosphorylates Src on Y527 and also downregulates Lyn, Fyn and Lck by tyrosine phosphorylation of carboxy terminal regulatory sites.
Catalog Number:
(10252-870)
Supplier:
Bioss
Description:
This gene encodes the acidic form of complement factor 4, part of the classical activation pathway. The protein is expressed as a single chain precursor which is proteolytically cleaved into a trimer of alpha, beta, and gamma chains prior to secretion. The trimer provides a surface for interaction between the antigen-antibody complex and other complement components. The alpha chain may be cleaved to release C4 anaphylatoxin, a mediator of local inflammation. Deficiency of this protein is associated with systemic lupus erythematosus and type I diabetes mellitus. This gene localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. Varying haplotypes of this gene cluster exist, such that individuals may have 1, 2, or 3 copies of this gene. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Nov 2011].
Catalog Number:
(10261-068)
Supplier:
Bioss
Description:
Synaptotagmins are a large gene family of synaptic vesicle type III integral membrane proteins that function as regulators of both exocytosis and endocytosis and are involved in neurotransmitter secretion from small secretory vesicles. Synaptotagmin XIV, also known as SytXIV, is a 555 amino acid single-pass type III membrane protein belonging to the Synaptotagmin family. With the ability to form heterodimers, Synaptotagmin XIV mainly exists as a homodimer and contains two C2 domains, an N-terminal transmembrane domain and a putative fatty-acylation site. Synaptotagmin XIV is Ca2+-independent and may function in the trafficking and exocytosis of secretory vesicles to tissues outside the brain. Disruption of Synaptotagmin XIV may be affiliated with neurodevelopmental abnormalities. Synaptotagmin XIV exists as six alternatively spliced isoforms and is encoded by a gene on human chromosome 1q32.2.
Catalog Number:
(10269-516)
Supplier:
Bioss
Description:
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 seven structurally related subunits designated GluR-1 to -7. The kainate/AMPA receptors are primarily responsible for the fast excitatory neuro-transmission by glutamate whereas the NMDA receptors are functionally characterized by a slow kinetic and a high permeability for Ca2+ ions. The NMDA receptors consist of five subunits: epsilion 1, 2, 3, 4 and one zeta subunit. The zeta subunit is expressed throughout the brainstem whereas the four epsilon subunits display limited distribution.
Catalog Number:
(10283-490)
Supplier:
Bioss
Description:
Stanniocalcin 1 (STC1) and stanniocalcin 2 (STC2) are mammalian peptide hormones that were previously considered to be present only in bony fish, where they are involved in calcium homeostasis. STC1 plays a role in calcium and phosphate homoeostasis and is phosphorylated in vitro by protein kinase C, and STC2 is phosphorylated in vitro by casein kinase II (CK2). A human fibrosarcoma cell line, HT1080, expresses both STC1 and STC2 as secreted phosphoproteins in vivo, with STC2 being phosphorylated by an ecto-CK2-like enzyme. STC1 and STC2 have opposite effects on calcium and phosphate homeostasis, namely anti-hypercalcemic and anti-hypocalcemic actions, respectively. STC1 and STC2 are detected in human adrenal tumors, such as pheochromocytoma, differentiated neuroblastoma aldosterone-producing adenoma, and in cultured adrenal tumor cells (rat pheochromocytoma PC-12 cells and human neuroblastoma NB-1 cells).
Catalog Number:
(10278-264)
Supplier:
Bioss
Description:
The PHEX a 749 amino acid protein that putatively consists of an intracellular, transmembrane and extracellular domain. PHEX mutations have been observed in 60-80% of hypophosphatemic rickets patients. The PHEX protein, which is a single-pass membrane protein, is also designated HYP, X-linked hypophosphatemia protein or metalloendopeptidase homolog PEX. PHEX plays an active role in bone and dentin mineralization and renal phosphate re-absorption. X-linked hypophosphatemic rickets, also designated HYP, is an X-linked dominant disorder characterized by impaired phosphate uptake in the kidney, which is likely to be caused by abnormal regulation of sodium phosphate cotransport in the proximal tubules. Clinical manifestations include skeletal deformities, growth failure, craniosynostosis, paravertebral calcifications, pseudofractures in lower extremities, and muscular hypotonia with onset in early childhood.
Catalog Number:
(10291-372)
Supplier:
Bioss
Description:
Filamins are Actin-binding proteins which contain an N-terminal Actin-binding domain, a membrane glycoprotein domain and a C-terminal self-association domain. Filamins help reshape the cytoskeleton by forming flexible cross-links between two Actin filaments, which maintain membrane integrity during force application. Filamins also participate in signal transduction pathways associated with cell motility, adhesion, differentiation and survival, and force transduction. The filamin family is comprised of Filamin 1, Filamin 2 and Filamin 3. Filamin 2, also designated Filamin C, is a skeletal- and cardiac-muscle specific form of Filamin, which binds ©-sarcoglycan and ∂-sarcoglycan, but not å-sarcoglycan or ∫-sarcoglycan. Muscular dystrophy, an inherited group of disorders resulting in progressive weakness of muscles in the body, is associated with irregular subcellular localization of Filamin 2 caused by a deficiency in KY, a protein that interacts with Filamin 2.
Catalog Number:
(10256-590)
Supplier:
Bioss
Description:
Rab 3, a neural/neuroendocrine-specific member of the Rab family, is involved in Ca2+-regulated exocytosis and functions in an inhibitory capacity, controlling the recruitment and pooling of secretory vesicles at the plasma membrane. The Rim (Rab 3 interacting molecule) family of proteins (Rim1, Rim2, Rim3 and Rim4) are multidomain adaptors that regulate Rab 3 activity and sub-sequent neurotransmitter release. Rim3, also known as RIMS3 (regulating synaptic membrane exocytosis 3) or NIM3, is a 308 amino acid member of the Rim family. Localized to the synapse and to cell junctions, Rim3 contains one C2 domain and is thought to play an important role in the regulation of synaptic membrane exocytosis. Rim3, a protein that may be phosphorylated upon DNA damage, is expressed throughout the body with highest levels present in brain tissue.
Catalog Number:
(10299-448)
Supplier:
Bioss
Description:
Transcription factors are required for the initiation of transcription. They regulate transcription by binding to DNA at specific nucleotide sequences within promoters and enhancers. Transcription factors, which may also bind to RNA polymerase or to other transcription factors, are involved in the preinitiation complex formation. Upstream transcription factors and inducible transcription factors bind upstream of the initiation site to repress or stimulate transcription. Upstream factors are unregulated, while inducible factors require inhibition or activation. GPBP1L1 (GC-rich promoter binding protein 1-like 1), also known as vasculin-like protein 1 or SP192, is a 474 amino acid protein belonging to the vasculin family. Localizing to nucleus, GPBP1L1 may function as a transcription factor. The gene encoding GPBP1L1 maps to human chromosome 1p34.1 and mouse chromosome 4 D1.
Catalog Number:
(10259-220)
Supplier:
Bioss
Description:
Sox genes comprise a family of genes that are related to the mammalian sex determining gene SRY. These genes similarly contain sequences that encode for the HMG-box domain, which is responsible for the sequence-specific DNA-binding activity. Sox genes encode putative transcriptional regulators implicated in the decision of cell fates during development and the control of diverse developmental processes. The highly complex group of Sox genes cluster at least 40 different loci that rapidly diverged in various animal lineages. At present, 30 Sox genes have been identified. Members of this family have been shown to be conserved during evolution and to play key roles during animal development. Some are involved in human diseases, including sex reversal.
Catalog Number:
(10303-902)
Supplier:
Bioss
Description:
Fyb (Fyn binding protein) and the anchoring proteins SKAP55 and SKAP55-R (SKAP55-related protein) associate with the tyrosine kinase p59fyn. SKAP55 and SKAP55-R bind to Fyb through their SH3 domains and function as substrates for p59Fyn in resting T cells. SKAP55 contains an N-terminal pleckstrin homology domain and a C-terminal SH3 domain binding motif of adjacent arginine and lysine residues followed by tandem tyrosines (i.e. RKxxYxxY). SKAP55-R, similar in overall structure to SKAP55, contains a coiled-coil N-terminal domain. SKAP55 associates with SLAP-130, another component of the Fyn complex, which plays a role in the regulation of signaling events initiated by lymphocyte antigen receptors leading up to T cell activation. The human SKAP55 gene maps to chromosome 17q21.32 and encodes a 359 amino acid protein.
Catalog Number:
(10281-484)
Supplier:
Bioss
Description:
Sorting nexin (SNX) proteins are members of a large family of hydrophilic PX (phospholipid-binding motif) domain-containing proteins that interact with a variety of receptor types. SNXs are widely expressed, although the tissue distribution of each SNX mRNA varies. The ability of SNXs to bind specific phospholipids, as well as their tendency to form protein-protein complexes, suggests a role for these proteins in cellular membrane trafficking and protein sorting. SNXs may also function specifically in pro-degradative sorting, internalization, endosomal recycling or simply in endosomal sorting. SNX10 (Sorting nexin-10) is a 201 amino acid protein that contains one phox domain and belongs to the SNX family. Like other members of the SNX family, SNX10 is thought to play a role in intracellular trafficking events throughout the cell.
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