Mouse Anti-ATP6AP1 Recombinant Antibody (CBT3011) (V2LY-0625-LY1741)

Name: Mouse Anti-ATP6AP1 Recombinant Antibody (CBT3011)
SKU: V2LY-0625-LY1741
Rating: 5 (1 reviews)

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Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

CBT3011

Antibody Isotype

IgG1

Application

WB, FC

Basic Information

Immunogen

Purified recombinant fragment of human ATP6AP1 (AA: 51-151) expressed in E. Coli.

Host Species

Mouse

Specificity

Human

Antibody Isotype

IgG1

Clonality

Monoclonal Antibody

Application Notes

Application	Note
WB	1:500-1:2,000
FC	1:200-1:400
ELISA	1:10,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Buffer

PBS

Preservative

Sodium azide

Concentration

Batch dependent

Purity

> 95% Purity determined by SDS-PAGE.

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name

ATPase H+ Transporting Accessory Protein 1

Entrez Gene ID

537

UniProt ID

Q15904

Function

Accessory subunit of the proton-transporting vacuolar (V)-ATPase protein pump, which is required for luminal acidification of secretory vesicles. Guides the V-type ATPase into specialized subcellular compartments, such as neuroendocrine regulated secretory vesicles or the ruffled border of the osteoclast, thereby regulating its activity. Involved in membrane trafficking and Ca2+-dependent membrane fusion. May play a role in the assembly of the V-type ATPase complex. In aerobic conditions, involved in intracellular iron homeostasis, thus triggering the activity of Fe2+ prolyl hydroxylase (PHD) enzymes, and leading to HIF1A hydroxylation and subsequent proteasomal degradation (PubMed:28296633).

Biological Process

Cellular iron ion homeostasis Source: UniProtKB
Cellular response to increased oxygen levels Source: UniProtKB
Establishment of organelle localization Source: UniProtKB
Insulin receptor signaling pathway Source: Reactome
Ion transmembrane transport Source: Reactome
pH reduction Source: UniProtKB
Positive regulation of bone resorption Source: UniProtKB
Positive regulation of ERK1 and ERK2 cascade Source: UniProtKB
Positive regulation of exocytosis Source: UniProtKB
Positive regulation of osteoblast differentiation Source: UniProtKB
Positive regulation of osteoclast development Source: UniProtKB
Proton transmembrane transport Source: ProtInc
Regulation of cellular pH Source: GO_Central
Transferrin transport Source: Reactome

Cellular Location

Endoplasmic reticulum membrane; Endoplasmic reticulum-Golgi intermediate compartment membrane. Not detected in trans-Golgi network.

Involvement in disease

Immunodeficiency 47 (IMD47): A complex immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infections, defective glycosylation of serum proteins, and liver disease with neonatal jaundice and hepatosplenomegaly. Some patients may also have neurologic features, including seizures, mild intellectual disability, and behavioral abnormalities. Inheritance is X-linked recessive.

Topology

Vacuolar: 42-419 aa
Helical: 420-440 aa
Cytoplasmic: 441-470 aa

PTM

N-glycosylated.

More Infomation

References

Wang, J., Liu, Y., & Zhang, S. (2021). Prognostic and immunological value of ATP6AP1 in breast cancer: implications for SARS-CoV-2. Aging (Albany NY), 13(13), 16904.

Tvina, A., Thomsen, A., & Palatnik, A. (2020). Prenatal and postnatal phenotype of a pathologic variant in the ATP6AP1 gene. European journal of medical genetics, 63(6), 103881.

Ondruskova, N., Honzik, T., Vondrackova, A., Stranecky, V., Tesarova, M., Zeman, J., & Hansikova, H. (2020). Severe phenotype of ATP6AP1‐CDG in two siblings with a novel mutation leading to a differential tissue‐specific ATP6AP1 protein pattern, cellular oxidative stress and hepatic copper accumulation. Journal of inherited metabolic disease, 43(4), 694-700.

Dimitrov, B., Himmelreich, N., Ederveen, A. L. H., Lüchtenborg, C., Okun, J. G., Breuer, M., ... & Thiel, C. (2018). Cutis laxa, exocrine pancreatic insufficiency and altered cellular metabolomics as additional symptoms in a new patient with ATP6AP1-CDG. Molecular genetics and metabolism, 123(3), 364-374.

Dimitrov, B. (2017). Klinische, molekularbiologische und biochemische Charakterisierung eines neuen Patientendefekts im „ATPase H+-transporting accessory protein 1 “(ATP6AP1)-Gen (Doctoral dissertation).

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Protocols

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Associated Products

Antibody Pairs

ATP6AP1 Matched Antibody Pair (117)

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Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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Documents

Datasheet
SDS
Request for COA

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Second antibody and isotype control

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