Hermansky Pudlak Syndrome Protein 4 (HPS4)
LE; Light-ear protein homolog
Hermansky-Pudlak syndrome is a disorder of organelle biogenesis in which oculocutaneous albinism, bleeding, and pulmonary fibrosis result from defects of melanosomes, platelet dense granules, and lysosomes. Mutations in this gene as well as several others can cause this syndrome.
HPS4appears to be important in organelle biogenesis and is similar to the mouse 'light ear' protein. Five transcript variants encoding different isoforms have been found for this gene. In addition, transcript variants utilizing alternative polyadenylation signals exist.In addition to their identical phenotypes, le and ep mutant mice have identical abnormalities of melanosomes, and in transfected melanoma cells, the HPS4 and HPS1 proteins partially colocalize in vesicles of the cell body.
Organism species: Homo sapiens (Human)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPM789Hu01 | Recombinant Hermansky Pudlak Syndrome Protein 4 (HPS4) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAM789Hu01 | Monoclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IP. |
| PAM789Hu01 | Polyclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IF. | |
| Assay Kits | CLM789Hu01 | CLIA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | CLIA assay for Antigen Detection |
| ELM789Hu01 | ELISA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Mus musculus (Mouse)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPM789Mu01 | Recombinant Hermansky Pudlak Syndrome Protein 4 (HPS4) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAM789Mu01 | Monoclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IP. |
| PAM789Mu01 | Polyclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IF. | |
| Assay Kits | CLM789Mu01 | CLIA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | CLIA assay for Antigen Detection |
| ELM789Mu01 | ELISA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Rattus norvegicus (Rat)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPM789Ra01 | Recombinant Hermansky Pudlak Syndrome Protein 4 (HPS4) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAM789Ra01 | Monoclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IP. |
| PAM789Ra01 | Polyclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IF. | |
| Assay Kits | CLM789Ra01 | CLIA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | CLIA assay for Antigen Detection |
| ELM789Ra01 | ELISA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Canis familiaris; Canine (Dog)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPM789Ca01 | Recombinant Hermansky Pudlak Syndrome Protein 4 (HPS4) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAM789Ca01 | Monoclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IP. |
| PAM789Ca01 | Polyclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IF. | |
| Assay Kits | CLM789Ca01 | CLIA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | CLIA assay for Antigen Detection |
| ELM789Ca01 | ELISA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Sus scrofa; Porcine (Pig)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPM789Po01 | Recombinant Hermansky Pudlak Syndrome Protein 4 (HPS4) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAM789Po01 | Monoclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IP. |
| PAM789Po01 | Polyclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IF. | |
| Assay Kits | CLM789Po01 | CLIA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | CLIA assay for Antigen Detection |
| ELM789Po01 | ELISA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Bos taurus; Bovine (Cattle)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPM789Bo01 | Recombinant Hermansky Pudlak Syndrome Protein 4 (HPS4) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAM789Bo01 | Monoclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IP. |
| PAM789Bo01 | Polyclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IF. | |
| Assay Kits | CLM789Bo01 | CLIA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | CLIA assay for Antigen Detection |
| ELM789Bo01 | ELISA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Oryctolagus cuniculus (Rabbit)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPM789Rb01 | Recombinant Hermansky Pudlak Syndrome Protein 4 (HPS4) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAM789Rb01 | Monoclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IP. |
| PAM789Rb01 | Polyclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IF. | |
| Assay Kits | CLM789Rb01 | CLIA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | CLIA assay for Antigen Detection |
| ELM789Rb01 | ELISA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | Enzyme-linked immunosorbent assay for Antigen Detection |
Organism species: Rhesus monkey (Simian)
| PRODUCT TYPE | CATALOG NO. | PRODUCT NAME | APPLICATIONS |
|---|---|---|---|
| Proteins | RPM789Mo01 | Recombinant Hermansky Pudlak Syndrome Protein 4 (HPS4) | Positive Control; Immunogen; SDS-PAGE; WB. |
| Antibodies | MAM789Mo01 | Monoclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IP. |
| PAM789Mo01 | Polyclonal Antibody to Hermansky Pudlak Syndrome Protein 4 (HPS4) | WB; IHC; ICC; IF. | |
| Assay Kits | CLM789Mo01 | CLIA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | CLIA assay for Antigen Detection |
| ELM789Mo01 | ELISA Kit for Hermansky Pudlak Syndrome Protein 4 (HPS4) | Enzyme-linked immunosorbent assay for Antigen Detection |
Reference
"Hermansky-Pudlak syndrome is caused by mutations in HPS4, the human homolog of the mouse light-ear gene."
Nat. Genet. 30:321-324(2002) [PubMed] [Europe PMC] [Abstract]
"Reevaluating human gene annotation: a second-generation analysis of chromosome 22."
Genome Res. 13:27-36(2003) [PubMed] [Europe PMC] [Abstract]
"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
"The full-ORF clone resource of the German cDNA consortium."
BMC Genomics 8:399-399(2007) [PubMed] [Europe PMC] [Abstract]
"The DNA sequence of human chromosome 22."
Nature 402:489-495(1999) [PubMed] [Europe PMC] [Abstract]
"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
"Identification of novel transcribed sequences on human chromosome 22 by expressed sequence tag mapping."
DNA Res. 8:1-9(2001) [PubMed] [Europe PMC] [Abstract]
"Phosphoproteome of resting human platelets."
J. Proteome Res. 7:526-534(2008) [PubMed] [Europe PMC] [Abstract]
"Quantitative phosphoproteomic analysis of T cell receptor signaling reveals system-wide modulation of protein-protein interactions."
Sci. Signal. 2:RA46-RA46(2009) [PubMed] [Europe PMC] [Abstract]
"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]