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Gene

PIGY

phosphatidylinositol glycan anchor biosynthesis class Y

Summary

Cytogenetic location
4q22.1
Coordinates
Chromosome 4: 88,520,998–88,523,776 (GRCh38)
Exons
2 exons across 2.8 kb
Protein
71 amino acids
Inheritance
Autosomal recessive
Also known as
MGC14156, PIG-Y
MANE Select transcript
ENST00000527353.2NM_001042616.3
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Reference genomeGRCh38

Positions on gene and variant pages follow this choice, in this browser.

Curated reports relevant to this gene

  • GPI Anchor Biosynthesis Defects

    • Hyperphosphatasia With Impaired Intellectual Development (Mabry Syndrome)Autosomal recessive

      A group of GPI anchor defects (PIGV, PIGO, PGAP2, PGAP3, PIGW, PIGY) in which a persistently high alkaline phosphatase accompanies developmental delay, seizures and short end bones of the fingers.

      • 1 GenCC submitter rates it definitive or strong
      What knowing changes

      Some affected children have had seizure control improved with pyridoxine (vitamin B6), so a diagnosis can guide a specialist trial of treatment.

    • Disorders of GPI Anchor Biosynthesis

      A group of inherited conditions caused by variants in the PIG, PGAP and related genes needed to make and attach the GPI anchor, typically causing developmental delay, low muscle tone and epilepsy.

      • 1 GenCC submitter rates it definitive or strong

Broader groupings

  • Congenital Disorders of Glycosylation

    • Congenital Disorders of Glycosylation (CDG)

      One of 98 genes linked to it

      A large group of inherited conditions in which sugar chains (glycans) are not correctly built or attached to proteins and lipids, often affecting the brain, liver, blood clotting, growth and many other body systems. Most are autosomal recessive, but some are X-linked or autosomal dominant.

      • 1 GenCC submitter rates it definitive or strong

Inheritance

Autosomal recessive
It takes a changed copy from each parent. With one, you are a carrier and usually unaffected; when both parents are carriers, each child has a one-in-four chance of inheriting two.

How PIGY is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Stretches short reads find hard
Chromosome 4, with a bracket where PIGY sits. PIGY and 10 genes near it on chromosome 4, from 88,022,387 to 89,022,386.Chromosome 44q22.1p16.3p16.2p16.1p15.33p15.32p15.31p15.2p15.1p14p13p12p11q11q12q13.1q13.2q13.3q21.1q21.21q21.22q21.23q21.3q22.1q22.2q22.3q23q24q25q26q27q28.1q28.2q28.3q31.1q31.21q31.22q31.23q31.3q32.1q32.2q32.3q33q34.1q34.2q34.3q35.1q35.288.2 Mb88.4 Mb88.6 Mb88.8 Mb89 MbPKD2: 88,007,586–88,077,777, forward strandPKD2ABCG2: 88,088,127–88,231,818, reverse strandABCG2PPM1K: 88,257,620–88,284,769, reverse strandPPM1KHERC6: 88,378,734–88,443,098, forward strandHERC6HERC5: 88,457,086–88,506,188, forward strandHERC5PYURF: 88,520,986–88,523,794, reverse strandPYURFPIGY: 88,520,998–88,523,776, reverse strandPIGYHERC3: 88,523,810–88,708,450, forward strandHERC3HERC3: 88,592,372–88,708,540, forward strandHERC3NAP1L5: 88,695,913–88,697,829, reverse strandNAP1L5FAM13A: 88,725,893–89,111,398, reverse strandFAM13ADiagram of PIGY: 2 exons on the reverse strand of chromosome 4Exon 2: 88,520,998–88,522,029, 1,032 basesExon 1: 88,523,498–88,523,776, 279 bases215′3′

Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.

Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).

Nearby genes from Ensembl release 116, on GRCh38.

Exons make up 1.3 kb of the gene's 2.8 kb; 216 of those bases code for the protein.

Where this comes from

  • HGNC: the gene's symbol, name, other symbols and location.
  • MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
  • GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
  • The ACMG secondary findings list, SF v3.2.
  • The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
  • Data release 2026.10.16-c6eea6bf.

Cite this page

APA

Dante Labs. (n.d.). PIGY gene: phosphatidylinositol glycan anchor biosynthesis class Y. Retrieved 17 October 2026, from https://dantelabs.com/genes/pigy

Vancouver

Dante Labs. PIGY gene: phosphatidylinositol glycan anchor biosynthesis class Y [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/pigy

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