BBS3; RP55

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

The antibody detects endogenous levels of total ARL6 protein.

Antigen affinity purification.

Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.

0.7 mg/ml (lot specific)

Store at -20 degree C

Small volumes of anti-ARL6 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

The protein encoded by this gene belongs to the ARF family of GTP-binding proteins. ARF proteins are important regulators of cellular traffic and are the founding members of an expanding family of homologous proteins and genomic sequences. They depart from other small GTP-binding proteins by a unique structural device that implements front-back communication from the N-terminus to the nucleotide-. Studies of the mouse ortholog of this protein suggest an involvement in protein transport, membrane trafficking, or cell signaling during hematopoietic maturation. Alternative splicing occurs at this locus and two transcript variants encoding the same protein have been described.

Total protein Ab

Immunohistochemistry (IHC)

Immunohistochemistry: 1:25-1:100

Immunohistochemical analysis of paraffin-embedded Human thyroid cancer tissue using at dilution 1/25.

21,960 Da

ADP-ribosylation factor-like 6

ADP-ribosylation factor-like protein 6

ADP-ribosylation factor-like protein 6

BBS3??[Similar Products]

ARL6_HUMAN

The protein encoded by this gene belongs to the ARF-like (ADP ribosylation factor-like) sub-family of the ARF family of GTP-binding proteins which are involved in regulation of intracellular traffic. Mutations in this gene are associated with Bardet-Biedl syndrome (BBS). Alternative splicing results in multiple transcript variants of this gene. A vision-specific transcript encoding a different protein has been described (PMID: 20333246). [provided by RefSeq, May 2013]

ARL6: Involved in membrane protein trafficking at the base of the ciliary organelle. Mediates recruitment onto plasma membrane of the BBSome complex which would constitute a coat complex required for sorting of specific membrane proteins to the primary cilia. May regulate cilia assembly and disassembly and subsequent ciliary signaling events such as the Wnt signaling cascade. Isoform 2 may be required for proper retinal function and organization. Defects in ARL6 are a cause of Bardet-Biedl syndrome type 3 (BBS3). Bardet-Biedl syndrome (BBS) is a genetically heterogeneous disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. Defects in ARL6 are the cause of retinitis pigmentosa type 55 (RP55). RP55 is a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. Belongs to the small GTPase superfamily. Arf family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: G protein, monomeric; G protein, monomeric, ARF; G protein

Chromosomal Location of Human Ortholog: 3q11.2

Cellular Component: membrane coat; membrane; cytoplasm; plasma membrane; axoneme; cytosol; cilium

Molecular Function: protein binding; GTP binding; metal ion binding; phospholipid binding

Biological Process: protein polymerization; fat cell differentiation; Wnt receptor signaling pathway; visual perception; organelle organization and biogenesis; small GTPase mediated signal transduction; cilium biogenesis; brain development; protein targeting to membrane; determination of left/right symmetry; intermembrane transport; melanosome transport; regulation of smoothened signaling pathway

Disease: Retinitis Pigmentosa 55; Bardet-biedl Syndrome 3; Bardet-biedl Syndrome 1; Retinitis Pigmentosa

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