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Showing 1201 to 1228 of 1228 found results for "Defective ALG14 Causes Congenital Myasthenic Syndrome (ALG14-CMS) Pathway"
Western Blot (WB)

OST48 Antibody

(#MBS9520518)

Gene Names:
OST48; DDOST; OST; WBP1; AGER1; CDG1R; OKSWcl45
Other Names:
dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit (non-catalytic)
Host/Reactivities:
Host: Rabbit / Reactivity: Human, Mouse, Rat
Purification:
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Isotype/Clone:
Isotype: Rabbit IgG
Applications:
Western Blot (WB)
  • Western Blot (WB)
  • Immunohistochemistry (IHC)
  • Immunohistochemistry (IHC)
  • Immunohistochemistry (IHC)
  • Immunohistochemistry (IHC)
  • Immunohistochemistry (IHC)
  • Immunohistochemistry (IHC)
  • Immunohistochemistry (IHC)
  • Flow Cytometry (FC/FACS)

    Anti-NANP Antibody Picoband

    (#MBS1759047)

    Gene Names:
    NANP; HDHD4; C20orf147; dJ694B14.3
    Other Names:
    one cut homeobox 2; One cut domain family member 2; Hepatocyte nuclear factor 6-beta; HNF-6-beta; Transcription factor ONECUT-2; OC-2; ONECUT2; HNF6B; N-acylneuraminate-9-phosphatase; N-acetylneuramin...
    Host/Reactivities:
    Host: Rabbit / Reactivity: Human, Mouse, Rat
    Purification:
    Immunogen affinity purified.
    Isotype/Clone:
    Isotype: Rabbit IgG
    Applications:
    Western Blot (WB), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS), ELISA (EIA)
    Western Blot (WB)

    MGAT3 Rabbit mAb

    (#MBS9148050)

    Gene Names:
    MGAT3
    Other Names:
    GNT3; GNT-III
    Host/Reactivities:
    Host: Rabbit / Reactivity: Human
    Purification:
    Affinity purification
    Isotype/Clone:
    Isotype: IgG
    Applications:
    Western Blot (WB), ELISA (EIA)
    GNPNAT1

    GNPNAT1 Antibody

    (#MBS9522289)

    Gene Names:
    GNPNAT1; GNPNAT; Gpnat1
    Other Names:
    Glucosamine 6-phosphate N-acetyltransferase (EC 2.3.1.4)(Phosphoglucosamine acetylase)(Phosphoglucosamine transacetylase); glucosamine 6-phosphate N-acetyltransferase; glucosamine-phosphate N-acetyltr...
    Host/Reactivities:
    Host: Rabbit / Reactivity: Human, Mouse
    Purification:
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
    Isotype/Clone:
    Isotype: Rabbit IgG
    Applications:
    Western Blot (WB)
    ST3GAL4

    ST3GAL4 Antibody

    (#MBS9522154)

    Gene Names:
    ST3GAL4; STZ; SAT3; CGS23; SIAT4; NANTA3; SIAT4C; ST3GalIV
    Other Names:
    CMP-N-acetylneuraminate-beta-galactosamide-alpha-2,3-sialyltransferase 4 (Alpha 2,3-ST 4)(Beta-galactoside alpha-2,3-sialyltransferase 4)(EC 2.4.99.-)(Alpha 2,3-sialyltransferase IV)(Gal-NAc6S)(Gal-be...
    Host/Reactivities:
    Host: Rabbit / Reactivity: Human, Mouse, Rat
    Purification:
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
    Isotype/Clone:
    Isotype: Rabbit IgG
    Applications:
    Western Blot (WB)
    PMM2

    PMM2 Antibody

    (#MBS9521776)

    Gene Names:
    PMM2; PMI; CDG1; CDGS; PMI1; CDG1a; PMM 2
    Other Names:
    Phosphomannomutase 2 (PMM 2)(EC 5.4.2.8); Phosphomannomutase 2
    Host/Reactivities:
    Host: Rabbit / Reactivity: Human, Mouse
    Purification:
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
    Isotype/Clone:
    Isotype: Rabbit IgG
    Applications:
    Western Blot (WB)
    • Immunohistochemistry (IHC)
    • Western Blot (WB)

      PMM1 Antibody

      (#MBS9520600)

      Gene Names:
      PMM1; Sec53
      Other Names:
      phosphomannomutase 1
      Host/Reactivities:
      Host: Rabbit / Reactivity: Human, Mouse
      Purification:
      The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
      Isotype/Clone:
      Isotype: Rabbit IgG
      Applications:
      Western Blot (WB), Immunohistochemistry (IHC)
      PGM3

      PGM3 Antibody

      (#MBS9523296)

      Gene Names:
      PGM3; AGM1; PAGM; IMD23; PGM 3
      Other Names:
      Phosphoacetylglucosamine mutase (PAGM)(EC 5.4.2.3)(Acetylglucosamine phosphomutase)(N-acetylglucosamine-phosphate mutase)(Phosphoglucomutase-3)(PGM 3); phosphoacetylglucosamine mutase isoform 1; phosp...
      Host/Reactivities:
      Host: Rabbit / Reactivity: Human, Mouse
      Purification:
      The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
      Isotype/Clone:
      Isotype: Rabbit IgG
      Applications:
      Western Blot (WB)
      Typical Testing Data/Standard Curve (for reference only)

      Human DDOST (Dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit) QuickTest ELISA Kit

      (#MBS7619880)

      Gene Names:
      DDOST; OST; WBP1; AGER1; CDG1R; OST48; OKSWcl45
      Other Names:
      Dolichyl-diphosphooligosaccharide--protein glycosyltransferase 48 kDa subunit; DDOST 48 kDa subunit; Oligosaccharyl transferase 48 kDa subunit; KIAA0115; dolichyl-diphosphooligosaccharide--protein gly...
      Host/Reactivities:
      Reactivity: Human
      Assay Type
      Sandwich ELISA, Double Antibody
      Samples
      Serum, plasma, cell culture supernatant, cell lysate or tissue lysate, other biological fluid samples
      Detection Range
      0.313-20ng/ml
      Sensitivity
      0.188ng/ml
      UGCGL1

      UGCGL1 Antibody

      (#MBS9521863)

      Gene Names:
      UGCGL1; UGGT1; UGT1; HUGT1
      Other Names:
      UDP-glucose:glycoprotein glucosyltransferase 1 (UGT1)(hUGT1)(EC 2.4.1.-)(UDP--Glc:glycoprotein glucosyltransferase)(UDP-glucose ceramide glucosyltransferase-like 1); UDP-glucose:glycoprotein glucosylt...
      Host/Reactivities:
      Host: Rabbit / Reactivity: Human, Mouse, Rat
      Purification:
      The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
      Isotype/Clone:
      Isotype: Rabbit IgG
      Applications:
      Western Blot (WB)
      • Western Blot (WB)
      • Immunohistochemistry (IHC)

        Recombinant Anti-UAP1 Rabbit mAb

        (#MBS8326978)

        Gene Names:
        UAP1; AGX; AGX1; AGX2; SPAG2
        Other Names:
        UDP-N-acetylhexosamine pyrophosphorylase; Antigen X; Sperm-associated antigen 2; UDP-N-acetylglucosamine pyrophosphorylase 1
        Host/Reactivities:
        Host: Rabbit / Reactivity: Human
        Purification:
        The antibody was purified by immunogen affinity chromatography.
        Applications:
        Western Blot (WB), Immunohistochemistry (IHC)
        RPN1

        RPN1 Antibody

        (#MBS9521744)

        Gene Names:
        RPN1; OST1; RBPH1
        Other Names:
        Dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 (EC 2.4.1.119)(Dolichyl-diphosphooligosaccharide--protein glycosyltransferase 67 kDa subunit)(Ribophorin I)(RPN-I)(Ribophorin-1...
        Host/Reactivities:
        Host: Rabbit / Reactivity: Human, Mouse, Rat
        Purification:
        The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
        Isotype/Clone:
        Isotype: Rabbit IgG
        Applications:
        Western Blot (WB)
        NANS

        NANS Antibody

        (#MBS9524257)

        Gene Names:
        NANS; SAS; HEL-S-100
        Other Names:
        Sialic acid synthase (N-acetylneuraminate synthase)(EC 2.5.1.56)(N-acetylneuraminate-9-phosphate synthase)(EC 2.5.1.57)(N-acetylneuraminic acid phosphate synthase)(N-acetylneuraminic acid synthase); s...
        Host/Reactivities:
        Host: Rabbit / Reactivity: Human
        Purification:
        The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
        Isotype/Clone:
        Isotype: Rabbit IgG
        Applications:
        Western Blot (WB)
        • Western Blot (WB)
        • Immunohistochemistry (IHC)

          Anti-MGAT3 Antibody Picoband

          (#MBS1758926)

          Gene Names:
          MGAT3; GNT3; GNT-III
          Other Names:
          lemur tyrosine kinase 3; Serine/threonine-protein kinase LMTK3; LMTK3; KIAA1883; TYKLM3; beta-1,4-mannosyl-glycoprotein 4-beta-N-acetylglucosaminyltransferase; mannosyl (beta-1,4-)-glycoprotein beta-1...
          Host/Reactivities:
          Host: Rabbit / Reactivity: Human
          Purification:
          Immunogen affinity purified.
          Isotype/Clone:
          Isotype: Rabbit IgG
          Applications:
          Western Blot (WB), Immunohistochemistry (IHC), ELISA (EIA)
          MAN2A1

          MAN2A1 Antibody

          (#MBS9523205)

          Gene Names:
          MAN2A1; MANA2; MANII; GOLIM7; AMan II
          Other Names:
          Alpha-mannosidase 2 (EC 3.2.1.114)(Golgi alpha-mannosidase II)(AMan II)(Man II)(Mannosidase alpha class 2A member 1)(Mannosyl-oligosaccharide 1,3-1,6-alpha-mannosidase); alpha-mannosidase 2; mannosida...
          Host/Reactivities:
          Host: Rabbit / Reactivity: Human, Mouse, Rat
          Purification:
          The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
          Isotype/Clone:
          Isotype: Rabbit IgG
          Applications:
          Western Blot (WB)
          GMPPB

          GMPPB Antibody

          (#MBS9522904)

          Gene Names:
          GMPPB; MDDGA14; MDDGB14; MDDGC14
          Other Names:
          Mannose-1-phosphate guanyltransferase beta (EC 2.7.7.13)(GDP-mannose pyrophosphorylase B)(GTP-mannose-1-phosphate guanylyltransferase beta); mannose-1-phosphate guanyltransferase beta isoform 1; GDP-m...
          Host/Reactivities:
          Host: Rabbit / Reactivity: Human, Mouse
          Purification:
          The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
          Isotype/Clone:
          Isotype: Rabbit IgG
          Applications:
          Western Blot (WB)
          FUT8

          FUT8 Antibody

          (#MBS9521892)

          Gene Names:
          FUT8
          Other Names:
          Alpha-(1,6)-fucosyltransferase (alpha1-6FucT)(EC 2.4.1.68)(Fucosyltransferase 8)(GDP-L-Fuc:N-acetyl-beta-D-glucosaminide alpha1,6-fucosyltransferase)(GDP-fucose--glycoprotein fucosyltransferase)(Glyco...
          Host/Reactivities:
          Host: Rabbit / Reactivity: Human, Mouse, Rat
          Purification:
          The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
          Isotype/Clone:
          Isotype: Rabbit IgG
          Applications:
          Western Blot (WB)
          Western Blot (WB)

          ALG1 Antibody

          (#MBS9519596)

          Gene Names:
          ALG1; HMT1; MT-1; CDG1K; HMAT1; HMT-1; Mat-1; hMat-1
          Other Names:
          Chitobiosyldiphosphodolichol beta-mannosyltransferase
          Host/Reactivities:
          Host: Rabbit / Reactivity: Human, Mouse
          Purification:
          The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
          Isotype/Clone:
          Isotype: Rabbit IgG
          Applications:
          Western Blot (WB)
          Western Blot (WB)

          ST3GAL4 Rabbit pAb

          (#MBS9148560)

          Gene Names:
          ST3GAL4
          Other Names:
          CGS23; NANTA3; SAT3; SIAT4; SIAT4C; ST-4; ST3GalA.2; ST3GalIV; STZ; gal-NAc6S; ST3 beta-galactoside alpha-2; 3-sialyltransferase 4
          Host/Reactivities:
          Host: Rabbit / Reactivity: Human
          Purification:
          Affinity purification
          Isotype/Clone:
          Isotype: IgG
          Applications:
          Western Blot (WB), ELISA (EIA)
          ST3GAL2

          ST3GAL2 Antibody

          (#MBS9524361)

          Gene Names:
          ST3GAL2; SIAT4B; ST3GALII; Gal-NAc6S; ST3GalA.2
          Other Names:
          CMP-N-acetylneuraminate-beta-galactosamide-alpha-2,3-sialyltransferase 2 (Alpha 2,3-ST 2)(Beta-galactoside alpha-2,3-sialyltransferase 2)(EC 2.4.99.4)(Gal-NAc6S)(Gal-beta-1,3-GalNAc-alpha-2,3-sialyltr...
          Host/Reactivities:
          Host: Rabbit / Reactivity: Human, Mouse, Rat
          Purification:
          The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
          Isotype/Clone:
          Isotype: Rabbit IgG
          Applications:
          Western Blot (WB)
          MGAT4A

          MGAT4A Antibody

          (#MBS9524119)

          Gene Names:
          MGAT4A; GNT-IV; GnT-4a; GNT-IVA
          Other Names:
          Alpha-1,3-mannosyl-glycoprotein 4-beta-N-acetylglucosaminyltransferase A (EC 2.4.1.145)(N-glycosyl-oligosaccharide-glycoprotein N-acetylglucosaminyltransferase IVa)(GlcNAc-T IVa)(GnT-IVa)(N-acetylgluc...
          Host/Reactivities:
          Host: Rabbit / Reactivity: Human, Mouse, Rat
          Purification:
          The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
          Isotype/Clone:
          Isotype: Rabbit IgG
          Applications:
          Western Blot (WB)
          GFPT2

          GFPT2 Antibody

          (#MBS9523579)

          Gene Names:
          GFPT2; GFAT2
          Other Names:
          Glucosamine--fructose-6-phosphate aminotransferase [isomerizing] 2 (EC 2.6.1.16)(D-fructose-6-phosphate amidotransferase 2)(Glutamine:fructose 6 phosphate amidotransferase 2)(GFAT 2)(GFAT2)(Hexosephos...
          Host/Reactivities:
          Host: Rabbit / Reactivity: Human, Mouse, Rat
          Purification:
          The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
          Isotype/Clone:
          Isotype: Rabbit IgG
          Applications:
          Western Blot (WB)
          Western Blot (WB)

          GFPT1 Antibody

          (#MBS9520120)

          Gene Names:
          GFPT1; GFA; GFAT; GFPT; MSLG; GFAT1; CMSTA1; GFAT 1; GFAT1m; GFPT1L
          Other Names:
          Glutamine--fructose-6-phosphate aminotransferase; glutamine--fructose-6-phosphate transaminase 1
          Host/Reactivities:
          Host: Rabbit / Reactivity: Human, Mouse, Rat
          Purification:
          The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
          Isotype/Clone:
          Isotype: Rabbit IgG
          Applications:
          Western Blot (WB)
          Dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1

          Human Dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit DAD1 ELISA Kit

          (#MBS2909771)

          Gene Names:
          DAD1; OST2
          Other Names:
          Defender against cell death 1; DAD-1; Oligosaccharyl transferase subunit DAD1; DAD1_HUMAN
          Host/Reactivities:
          Reactivity: Human
          Assay Type
          Sandwich
          Detection Range
          78-5000pg/mL
          Sensitivity
          30pg/mL
          Dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1

          Human Dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1 ELISA Kit

          (#MBS2910899)

          Gene Names:
          RPN1; OST1; RBPH1
          Other Names:
          Dolichyl-diphosphooligosaccharide--protein glycosyltransferase 67 kDa subunit; Ribophorin I; Ribophorin-1; RPN-I; RPN1_HUMAN ; dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit 1
          Host/Reactivities:
          Reactivity: Human
          DPM1

          DPM1 Antibody

          (#MBS9522657)

          Gene Names:
          DPM1; MPDS; CDGIE
          Other Names:
          Dolichol-phosphate mannosyltransferase (EC 2.4.1.83)(Dolichol-phosphate mannose synthase)(DPM synthase)(Dolichyl-phosphate beta-D-mannosyltransferase)(Mannose-P-dolichol synthase)(MPD synthase); dolic...
          Host/Reactivities:
          Host: Rabbit / Reactivity: Human, Mouse
          Purification:
          The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
          Isotype/Clone:
          Isotype: Rabbit IgG
          Applications:
          Western Blot (WB)
          Alpha-N-acetylgalactosaminide alpha-2,6-sialyltransferase 1

          Human Alpha-N-acetylgalactosaminide alpha-2,6-sialyltransferase 1 ELISA Kit

          (#MBS2911769)

          Gene Names:
          ST6GALNAC1; STYI; SIAT7A; HSY11339; ST6GalNAcI
          Other Names:
          GalNAc alpha-2; 6-sialyltransferase I; ST6GalNAc I; Sialyltransferase 7A; SIAT7-A; UNQ543/PRO848; SIA7A_HUMAN ; alpha-N-acetylgalactosaminide alpha-2,6-sialyltransferase 1 isoform 1; ST6 (alpha-N-acet...
          Host/Reactivities:
          Reactivity: Human
          Assay Type
          Sandwich
          Detection Range
          0.156-10ng/mL
          Sensitivity
          0.053ng/mL
          Alpha-1,3-mannosyl-glycoprotein 4-beta-N-acetylglucosaminyltransferase A

          Human Alpha-1,3-mannosyl-glycoprotein 4-beta-N-acetylglucosaminyltransferase A ELISA Kit

          (#MBS2910885)

          Gene Names:
          MGAT4A; GNT-IV; GnT-4a; GNT-IVA
          Other Names:
          N-glycosyl-oligosaccharide-glycoprotein N-acetylglucosaminyltransferase IVa; UDP-N-acetylglucosamine: alpha-1; 3-D-mannoside beta-1; 4-N-acetylglucosaminyltransferase IVa; GlcNAc-T IVa; MGT4A_HUMAN ; ...
          Host/Reactivities:
          Reactivity: Human
          Assay Type
          Sandwich
          Detection Range
          0.312-20ng/mL
          Sensitivity
          0.146ng/mL

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