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Official Full Name Bardet-Biedl syndrome 4
Background This gene is a member of the Bardet-Biedl syndrome (BBS) gene family. Bardet-Biedl syndrome is an autosomal recessive disorder characterized by severe pigmentary retinopathy, obesity, polydactyly, renal malformation and mental retardation. The proteins encoded by BBS gene family members are structurally diverse. The similar phenotypes exhibited by mutations in BBS gene family members are likely due to the proteins shared roles in cilia formation and function. Many BBS proteins localize to the basal bodies, ciliary axonemes, and pericentriolar regions of cells. BBS proteins may also be involved in intracellular trafficking via microtubule-related transport. The protein encoded by this gene has sequence similarity to O-linked N-acetylglucosamine (O-GlcNAc) transferases in plants and archaebacteria and in human forms a multi-protein "BBSome" complex with six other BBS proteins. Alternative splice variants have been described but their predicted protein products have not been experimentally verified.
Synonyms BBS4; Bardet-Biedl syndrome 4; Bardet-Biedl syndrome 4 protein; Bardet Biedl syndrome 4 protein; BBS4_HUMAN
    • Species :
    • Chicken
    • Human
    • Mouse
    • Zebrafish
    • Source :
    • E.coli
    • HEK293
    • HEK293T
    • Mammalian Cell
    • Wheat Germ
    • Tag :
    • GST
    • His
    • MYC/DDK
    • N/A
    Species Cat.# Product name Source (Host) Tag Protein Length Price
    Human BBS4-10155H Recombinant Human BBS4, GST-tagged E.coli GST
    Human BBS4-108H Recombinant Human BBS4 Protein, GST-tagged Wheat Germ GST
    Human BBS4-3959H Recombinant Human BBS4 Protein, Myc/DDK-tagged, C13 and N15-labeled HEK293T Myc/DDK
    Human BBS4-8502HCL Recombinant Human BBS4 293 Cell Lysate HEK293 N/A
    Mouse Bbs4-220M Recombinant Mouse Bbs4 Protein, MYC/DDK-tagged HEK293T MYC/DDK
    Chicken BBS4-4856C Recombinant Chicken BBS4 Mammalian Cell His
    Zebrafish BBS4-4740Z Recombinant Zebrafish BBS4 Mammalian Cell His

    BBS4 involved in several pathways and played different roles in them. We selected most pathways BBS4 participated on our site, such as Assembly of the primary cilium, BBSome-mediated cargo-targeting to cilium, Cargo trafficking to the periciliary membrane, which may be useful for your reference. Also, other proteins which involved in the same pathway with BBS4 were listed below. Creative BioMart supplied nearly all the proteins listed, you can search them on our site.

    Pathway Name Pathway Related Protein
    Assembly of the primary cilium FGFR1OP; NPHP1; CEP97; CCT4; MKKS; IFT20; NEK2; KIF3A; ODF2B; B9D2
    BBSome-mediated cargo-targeting to cilium PROL1; BBS1; BBS2; SETD6; BBS7; BBS10; LZTFL1; BBS9; CCT5; CCT8
    Cargo trafficking to the periciliary membrane MKKS; EXOC6; BBS10; CNGA2; UNC119B; BBS1; ARF4A; BBS9; PROL1; CCT2
    Organelle biogenesis and maintenance IFT43; MRPS27; WDR34; MRPS31; TTBK2; IFT20; NPHP1; PKD1; MRPS33; FBF1

    BBS4 has several biochemical functions, for example, RNA polymerase II repressing transcription factor binding, alpha-tubulin binding, beta-tubulin binding. Some of the functions are cooperated with other proteins, some of the functions could acted by BBS4 itself. We selected most functions BBS4 had, and list some proteins which have the same functions with BBS4. You can find most of the proteins on our site.

    Function Related Protein
    RNA polymerase II repressing transcription factor binding PROL1; TTC8; RBPJ; BBS2; SIN3A; MKKS; STAT3; TCF7L2; SP1; MTA1
    alpha-tubulin binding NDEL1; NCALD; PROL1; CAV3; PPARGC1A; HDAC6; PACRG; SPAST; DDL-2; FAM110C
    beta-tubulin binding EMD; UXT; PROL1; SPAST; RGS2; MAP1S; AKAP1; TBCD; SLC6A2; LRPPRC
    dynactin binding CCDC64; PROL1; BICD1; SNX5; SNX6; HTT; BBS4
    microtubule motor activity DNAI2; DNAH14; BBS4; KIF21A; KLC2; KIFC2; KIF14; DNAHC8; KIF12; KLC1B
    protein binding PARVA; HTR6; CDC26; CWC15; PKN3; BCR; IFITM3; PIK3R4; CD200R1; TA

    BBS4 has direct interactions with proteins and molecules. Those interactions were detected by several methods such as yeast two hybrid, co-IP, pull-down and so on. We selected proteins and molecules interacted with BBS4 here. Most of them are supplied by our site. Hope this information will be useful for your research of BBS4.

    PCM1; BBS1

    Hudak, LM; Lunt, S; et al. The Intraflagellar Transport Protein Ift80 Is Essential for Photoreceptor Survival in a Zebrafish Model of Jeune Asphyxiating Thoracic Dystrophy. INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE 51:3792-3799(2010).

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