PRODUCT CODE: ET7107-57

Recombinant Niemann Pick C1 Monoclonal Antibody (ET7107-57)

  • Recombinant

Applications

  • WB

  • ICC

  • IF

  • IHC-P

  • FC

REACTIVITY

  • Human

  • Mouse

  • Rat

Western blot analysis of Niemann Pick C1 on SiHa cell lysate using anti-Niemann Pick C1 antibody at 1/500 dilution.
  • Western blot analysis of Niemann Pick C1 on SiHa cell lysate using anti-Niemann Pick C1 antibody at 1/500 dilution.
  • ICC staining Niemann Pick C1 in A549 cells (green). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.
  • ICC staining Niemann Pick C1 in HepG2 cells (green). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.
  • ICC staining Niemann Pick C1 in PC-3M cells (green). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.
  • Immunohistochemical analysis of paraffin-embedded human kidney tissue using anti-Niemann Pick C1 antibody. Counter stained with hematoxylin.
  • Immunohistochemical analysis of paraffin-embedded rat kidney tissue using anti-Niemann Pick C1 antibody. Counter stained with hematoxylin.
  • Immunohistochemical analysis of paraffin-embedded mouse testis tissue using anti-Niemann Pick C1 antibody. Counter stained with hematoxylin.
  • Flow cytometric analysis of SH-SY-5Y cells with Niemann Pick C1 antibody at 1/100 dilution (red) compared with an unlabelled control (cells without incubation with primary antibody; black). Alexa Fluor 488-conjugated goat anti rabbit IgG was used as the secondary antibody.
Western blot analysis of Niemann Pick C1 on SiHa cell lysate using anti-Niemann Pick C1 antibody at 1/500 dilution.

Applications

  • WB

  • ICC

  • IF

  • IHC-P

  • FC

REACTIVITY

  • Human

  • Mouse

  • Rat

SPECIFICATIONS

Product Type

Recombinant Rabbit monoclonal primary

Product Name

Recombinant Niemann Pick C1 Monoclonal Antibody (ET7107-57)

Immunogen

Recombinant protein

Host

Rabbit

Positive Control

SiHa, A549, HepG2, PC-3M, Rat kidney tissue, human kidney tissue, mouse testis tissue, SH-SY-5Y.

Conjugation

Unconjugated

Clonality

Monoclonal

Clone Number

JB87-33

PROPERTIES

Form

Liquid

Storage Condition

Store at +4C after thawing. Aliquot store at -20C or -80C. Avoid repeated freeze / thaw cycles.

Storage Buffer

1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.

Concentration

1 ug/ul

PURIFICATION

Protein A purified.

MOLECULAR WEIGHT

142 kDa

Isotype

IgG

APPLICATION DILUTION

  • WB

  • 1:500-1:1,000

  • ICC/IF

  • 1:50-1:200

  • IHC-P

  • 1:50-1:200

  • FC

  • 1:50-1:100

TARGET

UNIPROT #

PROTEIN NAME

Niemann Pick C1

SYNONYMS

Niemann Pick C1 protein precursor antibody; Niemann Pick disease, type C1 antibody; Niemann-Pick C1 protein antibody; NPC antibody; NPC1 antibody; NPC1_HUMAN antibody

SEQUENCE SIMILARITIES

Belongs to the patched family.

POST-TRANSLATIONAL MODIFICATION

N-glycosylated.

SUBCELLULAR LOCATION

Endosome. Lysosome.

FUNCTION

Cells obtain cholesterol via two distinct pathways, endogenous synthesis in the endoplasmic reticulum and exogenous uptake through the low-density lipoprotein (LDL) receptor pathway. NPC1 is a protein that resides in late endosomes and lysosomes and is involved in the intracellular trafficking of cholesterol. The human NPC1 gene maps to chromosome 18q11.2 and produces proteins which undergo N-glycosylation and are expressed in brain and liver. NPC1 contains a cysteine-rich domain, which is critical for proper protein function, but is highly mutated. Mutations in NPC1 result in Niemann-Pick disease type C (NPC), an autosomal recessive disease characterized by the accumulation of unesterified cholesterol in the endosomal/lysosomal system. The accumulation of cholesterol results in progressive neurodegeneration and death. More than 90% of cases of NPC are due to mutations in NPC1 and patients with NPC display multiple neurological symptoms, such as hepatosplenomegaly, ataxia, dystonia and dementia.