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Catalog Number: (77440-836)
Supplier: Bioss
Description: Chromosome 1 is the largest human chromosome spanning about 260 million base pairs and making up 8% of the human genome. There are about 3,000 genes on chromosome 1, and considering the great number of genes there are also a large number of diseases associated with chromosome 1. Notably, the rare aging disease Hutchinson-Gilford progeria is associated with the LMNA gene which encodes lamin A. When defective, the LMNA gene product can build up in the nucleus and cause characteristic nuclear blebs. The mechanism of rapidly enhanced aging is unclear and is a topic of continuing exploration. The MUTYH gene is located on chromosome 1 and is partially responsible for familial adenomatous polyposis. Stickler syndrome, Parkinsons, Gaucher disease and Usher syndrome are also associated with chromosome 1. A breakpoint has been identified in 1q which disrupts the DISC1 gene and is linked to schizophrenia. Aberrations in chromosome 1 are found in a variety of cancers including head and neck cancer, malignant melanoma and multiple myeloma. The FAM43B gene product has been provisionally designated FAM43B pending further characterization.


Catalog Number: (10109-064)
Supplier: Prosci
Description: PRPF19 plays a role in DNA double-strand break (DSB) repair and pre-mRNA splicing reaction. It binds double-stranded DNA in a sequence-nonspecific manner. PRPF19 acts as a structural component of the nuclear framework. It may also serve as a support for spliceosome binding and activity. It is essential for spliceosome assembly in a oligomerization-dependent manner and might also be important for spliceosome stability. It also may have E3 ubiquitin ligase activity. The PSO4 complex is required in the DNA interstrand cross-links (ICLs) repair process. Overexpression of PRPF19 might extend the cellular life span by increasing the resistance to stress or by improving the DNA repair capacity of the cells.In S. cerevisiae, Pso4 has pleiotropic functions in DNA recombination and in error-prone nonhomologous end-joining DNA repair.


Supplier: Biotium
Description: This antibody reacts with Ig domain of CD47 protein. It has been shown to inhibit polymorphonuclear neutrophil (PMN) transmigration across cell monolayers and matrix. CD47, originally named integrin-associated protein (IAP), is a 50 kDa protein containing five membrane-spanning sequences and a short cytoplasmic tail. CD47 plays a role in both cell adhesion by acting as an adhesion receptor for THBS1 on platelets, and in the modulation of integrins. It is important in memory formation and synaptic plasticity in the hippocampus. CD47 may play a role in membrane transport and/or integrin dependent signal transduction.

CF® dyes are Biotium's next-generation fluorescent dyes. CF®594 is a deep red fluorescent dye (Ex/Em 593/614 nm). It yields the brightest conjugates among spectrally similar dyes, and has excellent photostability.

Catalog Number: (75966-472)
Supplier: Biotium
Description: This antibody recognizes a protein of ~76 kDa, which is identified as Nucleolin (NCL). It is the major nucleolar phosphoprotein of growing eukaryotic cells. NCL is located mainly in dense fibrillar regions of the nucleolus. It is found associated with intranucleolar chromatin and pre-ribosomal particles. Human NCL gene consists of 14 exons with 13 introns and spans approximately 11kb. It induces chromatin decondensation by binding to histone H1. It is thought to play a role in pre-rRNA transcription and ribosome assembly.This MAb can be used to stain the nucleoli in cell or tissue preparations and can be used as a marker of the nucleoli in subcellular fractions. It produces a speckled pattern in the nuclei of cells of normal and malignant cells and may be used to stain the nucleoli of cells in fixed or frozen tissue sections. It can be used with paraformaldehyde fixed frozen tissue or cell preparations and formalin fixed, paraffin-embedded tissue sections.


Catalog Number: (76107-652)
Supplier: Bioss
Description: NHLRC2 (NHL repeat containing 2) is a 726 amino acid protein that contains six NHL repeats and one thioredoxin domain. Existing as two alternatively spliced isoforms, NHLRC2 is encoded by a gene that maps to human chromosome 10q25.3. Spanning nearly 135 million base pairs, chromosome 10 makes up approximately 4.5% of total DNA in cells and encodes nearly 1,200 genes. Several protein-coding genes, including those that encode for chemokines, cadherins, excision repair proteins, early growth response factors (Egrs) and fibroblast growth receptors (FGFRs), are located on chromosome 10. Defects in some of the genes that map to chromosome 10 are associated with Charcot-Marie Tooth disease, Jackson-Weiss syndrome, Usher syndrome, nonsyndromatic deafness, Wolman?s syndrome, Cowden syndrome, multiple endocrine neoplasia type 2 and porphyria.


Catalog Number: (10429-364)
Supplier: Bioss
Description: FGFRL1 is a member of the fibroblast growth factor receptor (FGFR) family, where amino acid sequence is highly conserved between members and throughout evolution. FGFR family members differ from one another in their ligand affinities and tissue distribution. A full-length representative protein would consist of an extracellular region, composed of three immunoglobulin-like domains, a single hydrophobic membrane-spanning segment and a cytoplasmic tyrosine kinase domain. The extracellular portion of the protein interacts with fibroblast growth factors, setting in motion a cascade of downstream signals, ultimately influencing mitogenesis and differentiation. A marked difference between FGFRL1 and the other family members is its lack of a cytoplasmic tyrosine kinase domain. The result is a transmembrane receptor that could interact with other family members and potentially inhibit signaling. Multiple alternatively spliced transcript variants encoding the same isoform have been found.


Catalog Number: (10748-668)
Supplier: Prosci
Description: ORAI3 Antibody: Antigen stimulation of immune cells triggers Ca++ entry through Ca++ release-activated Ca++ (CRAC) channels. ORAI3 is one of two mammalian homologs to ORAI1, a recently identified four-transmembrane spanning protein that is an essential component of CRAC. All three homologs have been shown to function as Ca++ plasma membrane channels gated through interactions with STIM1, the store-activated endoplasmic reticulum Ca++ sensor. However, ORAI3 channels failed to produce detectable Ca++ selective currents in cells co-transfected with ORAI3 and STIM1, indicating that ORAI3 channels undergo a lesser degree of depotentiation than ORAI1 or ORAI2. Na+ currents through ORAI1, 2 and 3 channels were equally inhibited by extracellular Ca++, indicating that each have similar affinities for Ca++ within the selectivity filter.


Catalog Number: (10483-358)
Supplier: Bioss
Description: The ubiquitin (Ub) pathway involves three sequential enzymatic steps that facilitate the conjugation of Ub and Ub-like molecules to specific protein substrates. The first step requires ATP-dependent activation of the Ub C-terminus and assembly of multi-Ub chains by a Ub-activating enzyme known as the E1 component. The Ub chain is then conjugated to the Ub-conjugating enzyme (E2) to generate an intermediate Ub-E2 complex. The Ub-ligase (E3) then catalyzes the transfer of Ub from E2 to the appropriate protein substrate. UBE2U (ubiquitin-conjugating enzyme E2U) is a 321 amino acid E2 ubiquitin conjugating enzyme that catalyzes the covalent attachment of ubiquitin to other proteins. Existing as two alternatively spliced isoforms, UBE2U is encoded by a gene located on human chromosome 1, which spans 260 million base pairs, contains over 3,000 genes and comprises nearly 8% of the human genome.


Catalog Number: (77436-808)
Supplier: Bioss
Description: Low density lipoprotein receptor-related protein (also known as low density LRP, LRP1, alpha-2-macroglobulin receptor or Apolipoprotein E receptor) is an endocytotic receptor that is involved both in endocytosis and in phagocytosis of apoptotic cells. It is required for early embryonic development, is involved in cellular lipid homeostasis, and may play a role in APP metabolism, kinase-dependent intracellular signalling, neuronal calcium signalling and neurotransmission. Low density LRP also plays a role in the plasma clearance of chylomicron remnants and activated LRPAP1 (alpha-2-macroglobulin), and is involved in the local metabolism of complexes of plasminogen activators and their endogenous ligands. Low density LPR is postulated to be one of the major players in host resistance to HIV. The precursor low density LRP molecule is cleaved post-translationally to form a 85 kDa membrane-spanning subunit (LRP-85) and a 515 kDa large extracellular domain (LRP-515), which remains non-covalently associated with LRP-85. Following cleavage, the intracellular domain (LPRICD) is present in both the cytoplasm and the nucleus.


Supplier: Biotium
Description: This antibody reacts with Ig domain of CD47 protein. It has been shown to inhibit polymorphonuclear neutrophil (PMN) transmigration across cell monolayers and matrix. CD47, originally named integrin-associated protein (IAP), is a 50 kDa protein containing five membrane-spanning sequences and a short cytoplasmic tail. CD47 plays a role in both cell adhesion by acting as an adhesion receptor for THBS1 on platelets, and in the modulation of integrins. It is important in memory formation and synaptic plasticity in the hippocampus. CD47 may play a role in membrane transport and/or integrin dependent signal transduction.

CF® dyes are Biotium's next-generation fluorescent dyes. CF®568 is a red fluorescent dye (Ex/Em 562/583 nm) with superior brightness and photostability. It also is compatible with super-resolution imaging by STORM and TIRF.

Catalog Number: (76109-946)
Supplier: Bioss
Description: The AAA ATPase family of molecular chaperones are characterized by a highly conserved AAA motif. Composed of 200-250 residues, the AAA domain contains Walker homology sequences and imparts ATPase activity. Members of the AAA ATPase family act as DNA helicases as well as transcription factors and are thought to be involved in several cellular functions such as cell-cycle regulation, protein proteolysis, organelle biogenesis and vesicle-mediated protein transport. Mitochondrial membrane proteins ATAD3A and ATAD3B contribute to the stabilization of nucleoids which are large mitochondrial DNA (mtDNA)-protein complexes. ATAD3A/B may participate in the transformation pathway and the chemosensitivity of oligodendrogliomas. The genes encoding ATAD3A/B/C maps to human chromosome 1, which houses over 3,000 genes and is the largest human chromosome spanning about 260 million base pairs and making up 8% of the human genome.


Catalog Number: (76108-686)
Supplier: Bioss
Description: The ubiquitin (Ub) pathway involves three sequential enzymatic steps that facilitate the conjugation of Ub and Ub-like molecules to specific protein substrates. The first step requires ATP-dependent activation of the Ub C-terminus and assembly of multi-Ub chains by a Ub-activating enzyme known as the E1 component. The Ub chain is then conjugated to the Ub-conjugating enzyme (E2) to generate an intermediate Ub-E2 complex. The Ub-ligase (E3) then catalyzes the transfer of Ub from E2 to the appropriate protein substrate. UBE2U (ubiquitin-conjugating enzyme E2U) is a 321 amino acid E2 ubiquitin conjugating enzyme that catalyzes the covalent attachment of ubiquitin to other proteins. Existing as two alternatively spliced isoforms, UBE2U is encoded by a gene located on human chromosome 1, which spans 260 million base pairs, contains over 3,000 genes and comprises nearly 8% of the human genome.


Catalog Number: (76107-654)
Supplier: Bioss
Description: NHLRC2 (NHL repeat containing 2) is a 726 amino acid protein that contains six NHL repeats and one thioredoxin domain. Existing as two alternatively spliced isoforms, NHLRC2 is encoded by a gene that maps to human chromosome 10q25.3. Spanning nearly 135 million base pairs, chromosome 10 makes up approximately 4.5% of total DNA in cells and encodes nearly 1,200 genes. Several protein-coding genes, including those that encode for chemokines, cadherins, excision repair proteins, early growth response factors (Egrs) and fibroblast growth receptors (FGFRs), are located on chromosome 10. Defects in some of the genes that map to chromosome 10 are associated with Charcot-Marie Tooth disease, Jackson-Weiss syndrome, Usher syndrome, nonsyndromatic deafness, Wolman?s syndrome, Cowden syndrome, multiple endocrine neoplasia type 2 and porphyria.


Catalog Number: (10429-360)
Supplier: Bioss
Description: FGFRL1 is a member of the fibroblast growth factor receptor (FGFR) family, where amino acid sequence is highly conserved between members and throughout evolution. FGFR family members differ from one another in their ligand affinities and tissue distribution. A full-length representative protein would consist of an extracellular region, composed of three immunoglobulin-like domains, a single hydrophobic membrane-spanning segment and a cytoplasmic tyrosine kinase domain. The extracellular portion of the protein interacts with fibroblast growth factors, setting in motion a cascade of downstream signals, ultimately influencing mitogenesis and differentiation. A marked difference between FGFRL1 and the other family members is its lack of a cytoplasmic tyrosine kinase domain. The result is a transmembrane receptor that could interact with other family members and potentially inhibit signaling. Multiple alternatively spliced transcript variants encoding the same isoform have been found.


Catalog Number: (10482-674)
Supplier: Bioss
Description: Kelch domain-containing protein 8A (KLHDC8A) is a 350 amino acid protein. KLHDC8A contains seven kelch repeats, each of which is an approximately 50 amino acid long conserved region that forms a tertiary structure beta-propeller. The gene that encodes KLHDC8A is located on chromosome 1, which is the largest human chromosome, spanning about 260 million base pairs and making up 8% of the human genome. There are about 3,000 genes on chromosome 1, and considering the great number of genes there are also a large number of diseases associated with chromosome 1. Notably, the rare aging disease Hutchinson-Gilford progeria is associated with the LMNA gene which encodes Lamin A. The MUTYH gene is located on chromosome 1 and is partially responsible for familial adenomatous polyposis. Stickler syndrome, Parkinsons, Gaucher disease and Usher syndrome are also associated with chromosome 1. Aberrations in chromosome 1 are found in a variety of cancers including head and neck cancer, malignant melanoma and multiple myeloma.


Catalog Number: (10482-676)
Supplier: Bioss
Description: Kelch domain-containing protein 8A (KLHDC8A) is a 350 amino acid protein. KLHDC8A contains seven kelch repeats, each of which is an approximately 50 amino acid long conserved region that forms a tertiary structure beta-propeller. The gene that encodes KLHDC8A is located on chromosome 1, which is the largest human chromosome, spanning about 260 million base pairs and making up 8% of the human genome. There are about 3,000 genes on chromosome 1, and considering the great number of genes there are also a large number of diseases associated with chromosome 1. Notably, the rare aging disease Hutchinson-Gilford progeria is associated with the LMNA gene which encodes Lamin A. The MUTYH gene is located on chromosome 1 and is partially responsible for familial adenomatous polyposis. Stickler syndrome, Parkinsons, Gaucher disease and Usher syndrome are also associated with chromosome 1. Aberrations in chromosome 1 are found in a variety of cancers including head and neck cancer, malignant melanoma and multiple myeloma.


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